Phenotypes associated with this allele

• Allele Symbol: Tg(GFAP-cre)25Mes
• Allele Name: transgene insertion 25, Albee Messing
• Allele ID: MGI:2179048
• Summary: 60 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Hk2^tm1.1Uku/Hk2^tm1.1Uku Tg(GFAP-cre)25Mes/0	B6.Cg-Hk2^tm1.1Uku Tg(GFAP-cre)25Mes	MGI:5571382
cn2	Mrtfa^tm1Eno/Mrtfa^tm1Eno Mrtfb^tm2.1Eno/Mrtfb^tm2.1Eno Tg(GFAP-cre)25Mes/0	involves: 129 * 129S/SvEv * C57BL/6 * FVB/N	MGI:4818692
cn3	Pdcd10^tm1Wami/Pdcd10^tm1Wami Tg(GFAP-cre)25Mes/0	involves: 129 * C57BL/6 * FVB/N	MGI:5002697
cn4	Epor^tm1Hwu/Epor^tm1Hwu Tg(GFAP-cre)25Mes/?	involves: 129 * C57BL/6 * FVB/N	MGI:3613565
cn5	Gt(ROSA)26Sor^tm1(RICTOR)Jger/Gt(ROSA)26Sor^tm1(RICTOR)Jger Tg(GFAP-cre)25Mes/0	involves: 129 * FVB/N	MGI:5472015
cn6	Knl1^tm1c(EUCOMM)Hmgu/Knl1^tm1c(EUCOMM)Hmgu Trp53^tm1Brn/Trp53^tm1Brn Tg(GFAP-cre)25Mes/0	involves: 129P2/OlaHsd * 129S4/SvJaeSor * C57BL/6N * FVB/N	MGI:6358254
cn7	Pten^tm1Rdp/Pten^+ Trp53^tm1Brn/Trp53^tm1Brn Tg(GFAP-cre)25Mes/0	involves: 129P2/OlaHsd * 129S6/SvEvTac * C57BL/6 * FVB/N	MGI:3831280
cn8	Gt(ROSA)26Sor^tm1(Smo/EYFP)Amc/Gt(ROSA)26Sor^+ Hk2^tm1.1Uku/Hk2^+ Tg(GFAP-cre)25Mes/0	involves: 129P2/OlaHsd * 129X1/SvJ * C57BL/6 * FVB/N	MGI:5571384
cn9	Gt(ROSA)26Sor^tm1(Smo/EYFP)Amc/Gt(ROSA)26Sor^+ Hk2^tm1.1Uku/Hk2^tm1.1Uku Tg(GFAP-cre)25Mes/0	involves: 129P2/OlaHsd * 129X1/SvJ * C57BL/6 * FVB/N	MGI:5571383
cn10	Mib1^tm2Kong/Mib1^tm2Kong Tg(GFAP-cre)25Mes/0	involves: 129P2/OlaHsd * C57BL/6 * FVB/N	MGI:3804817
cn11	Trp53^tm1Brn/Trp53^tm1Brn Tg(GFAP-cre)25Mes/0	involves: 129P2/OlaHsd * C57BL/6 * FVB/N	MGI:3831281
cn12	Glul^tm3Whla/Glul^tm3Whla Tg(GFAP-cre)25Mes/0	involves: 129P2/OlaHsd * FVB/N	MGI:4462797
cn13	Phgdh^tm1.2Shfu/Phgdh^tm1.2Shfu Tg(GFAP-cre)25Mes/0	involves: 129P2/OlaHsd * FVB/N	MGI:4881761
cn14	Cdc42^tm1.1Rac/Cdc42^tm1.1Rac Tg(GFAP-cre)25Mes/0	involves: 129P2/OlaHsd * FVB/N	MGI:5495325
cn15	Glul^tm3Whla/Glul^tm1Whla Tg(GFAP-cre)25Mes/0	involves: 129P2/OlaHsd * FVB/N	MGI:4462796
cn16	Nf1^tm1Par/Nf1^+ Pten^tm1Hwu/Pten^+ Trp53^tm1Tyj/Trp53^+ Tg(GFAP-cre)25Mes/0	involves: 129S1/Sv * 129S2/SvPas * 129S4/SvJae * 129X1/SvJ * FVB/N	MGI:4840094
cn17	Nf1^tm1Par/Nf1^+ Trp53^tm1Tyj/Trp53^+ Tg(GFAP-cre)25Mes/0	involves: 129S1/Sv * 129S2/SvPas * 129X1/SvJ * FVB/N	MGI:4840090
cn18	Nf1^tm1Par/Nf1^+ Pten^tm1Hwu/Pten^+ Trp53^tm1Elee/Trp53^+ Tg(GFAP-cre)25Mes/0	involves: 129S1/Sv * 129S4/SvJae * 129X1/SvJ * FVB/N	MGI:4840096
cn19	Nf1^tm1Par/Nf1^+ Trp53^tm1Elee/Trp53^+ Tg(GFAP-cre)25Mes/0	involves: 129S1/Sv * 129S4/SvJae * 129X1/SvJ * FVB/N	MGI:4840095
cn20	Mapk1^tm1Gela/Mapk1^tm1Gela Tg(GFAP-cre)25Mes/0	involves: 129S1/Sv * 129X1/SvJ * FVB/N	MGI:3805515
cn21	Dag1^tm1Kcam/Dag1^tm2Kcam Tg(GFAP-cre)25Mes/0	involves: 129S1/Sv * 129X1/SvJ * FVB/N	MGI:2684282
cn22	Dag1^tm2Kcam/Dag1^tm2Kcam Tg(GFAP-cre)25Mes/0	involves: 129S1/Sv * 129X1/SvJ * FVB/N	MGI:2684281
cn23	Gt(ROSA)26Sor^tm1(RICTOR)Jger/Gt(ROSA)26Sor^tm1(RICTOR)Jger Tg(GFAP-cre)25Mes/0 Tg(GFAP-EGFR*,-lacZ)#Agu/0	involves: 129S1/Sv * 129X1/SvJ * FVB/N	MGI:5472016
cn24	Ntf3^tm1Esm/Ntf3^tm1Esm Tg(GFAP-cre)25Mes/0	involves: 129S1/Sv * FVB/N	MGI:4436771
cn25	Trp53^tm1Elee/Trp53^tm1Tyj Tg(GFAP-cre)25Mes/0	involves: 129S2/SvPas * 129S4/SvJae * FVB/N	MGI:3849178
cn26	Kmt2a^tm1.1Erns/Kmt2a^tm1.1Erns Tg(GFAP-cre)25Mes/0	involves: 129S2/SvPas * FVB/N	MGI:3839880
cn27	Olig2^tm1Qrlu/Olig2^tm1Qrlu Tg(GFAP-cre)25Mes/?	involves: 129S4/SvJae * C57BL/6J * FVB/N	MGI:3615302
cn28	Trp53^tm1Elee/Trp53^tm1Elee Tg(GFAP-cre)25Mes/0	involves: 129S4/SvJae * FVB/N	MGI:3849177
cn29	Pten^tm1Hwu/Pten^tm1Hwu Tg(GFAP-cre)25Mes/0	involves: 129S4/SvJae * FVB/N	MGI:4830362
cn30	Pomt2^tm1.1Hhu/Pomt2^tm1.1Hhu Tg(GFAP-cre)25Mes/0	involves: 129S4/SvJaeSor * 129S6/SvEvTac * C57BL/6J * FVB/N	MGI:5302861
cn31	Knl1^tm1c(EUCOMM)Hmgu/Knl1^tm1c(EUCOMM)Hmgu Tg(GFAP-cre)25Mes/0	involves: 129S4/SvJaeSor * C57BL/6N * FVB/N	MGI:6358252
cn32	Cul3^tm1Jdsr/Cul3^+ Tg(GFAP-cre)25Mes/0	involves: 129S4/SvJaeSor * FVB/N	MGI:6449671
cn33	Cul3^tm1Jdsr/Cul3^tm1Jdsr Tg(GFAP-cre)25Mes/0	involves: 129S4/SvJaeSor * FVB/N	MGI:6449669
cn34	Nsdhl^tm1.1Hrm/Y Tg(GFAP-cre)25Mes/0	involves: 129S6/SvEvTac * C57BL/6J * FVB/N	MGI:5896534
cn35	Nf1^tm1Fcr/Nf1^tm1Fcr Trp53^tm1Elee/Trp53^tm1Elee Tg(GFAP-cre)25Mes/0	involves: 129S/SvEv * 129S4/SvJae * FVB/N	MGI:3849179
cn36	Mef2a^tm1.1Limm/Mef2a^tm1.1Limm Mef2d^tm3Eno/Mef2d^tm3Eno Tg(GFAP-cre)25Mes/0	involves: 129S/SvEv * C57BL/6 * FVB/N	MGI:5560659
cn37	Mrtfb^tm2.1Eno/Mrtfb^tm2.1Eno Tg(GFAP-cre)25Mes/0	involves: 129S/SvEv * C57BL/6 * FVB/N	MGI:4818691
cn38	Mef2a^tm1.1Limm/Mef2a^tm1.1Limm Mef2c^tm2Eno/Mef2c^tm2Eno Mef2d^tm3Eno/Mef2d^tm3Eno Tg(GFAP-cre)25Mes/0	involves: 129S/SvEv * C57BL/6 * FVB/N	MGI:5560657
cn39	Mef2a^tm1.1Limm/Mef2a^tm1.1Limm Tg(GFAP-cre)25Mes/0	involves: 129S/SvEv * C57BL/6 * FVB/N	MGI:5560660
cn40	Lgi1^tm2.1Jkc/Lgi1^tm2.1Jkc Tg(GFAP-cre)25Mes/0	involves: 129/Sv * C57BL/6 * FVB/N	MGI:6275806
cn41	Ptch1^tm1Bjw/Ptch1^tm1Bjw Tg(GFAP-cre)25Mes/0	involves: 129T2/SvEms * FVB/N	MGI:5286072
cn42	Gt(ROSA)26Sor^tm1(Smo/EYFP)Amc/Gt(ROSA)26Sor^tm1(Smo/EYFP)Amc Tg(GFAP-cre)25Mes/0	involves: 129X1/SvJ * FVB/N	MGI:3810317
cn43	Fgfr2^tm1Dor/Fgfr2^tm1Dor Tg(GFAP-cre)25Mes/0	involves: 129X1/SvJ * FVB/N	MGI:3641105
cn44	Fgfr1^tm1Upir/Fgfr1^tm1Upir Fgfr2^tm1Dor/Fgfr2^tm1Dor Tg(GFAP-cre)25Mes/0	involves: 129X1/SvJ * FVB/N	MGI:3641106
cn45	Bicd2^tm1Hgrd/Bicd2^tm1Hgrd Tg(GFAP-cre)25Mes/0	involves: C57BL/6 * FVB/N	MGI:5810136
cn46	Ephx2^tm1.1Arte/Ephx2^tm1.1Arte Tg(GFAP-cre)25Mes/0	involves: C57BL/6 * FVB/N	MGI:5609321
cn47	Hip1^tm5.1(HIP1)Tsr/Hip1^tm5.1(HIP1)Tsr Tg(GFAP-cre)25Mes/0	involves: C57BL/6 * FVB/N	MGI:6719557
cn48	Drd2^tm1Smoc/Drd2^tm1Smoc Tg(GFAP-cre)25Mes/0	involves: C57BL/6 * FVB/N	MGI:5499900
cn49	Trpv4^tm1c(KOMP)Wtsi/Trpv4^tm1c(KOMP)Wtsi Tg(GFAP-cre)25Mes/0	involves: C57BL/6J * C57BL/6N * FVB/N	MGI:6256831
cn50	Aqp4^tm1.1Geno/Aqp4^tm1.1Geno Tg(GFAP-cre)25Mes/0	involves: C57BL/6J * FVB/N	MGI:5297540
cn51	Marveld1^tm1.1Liy/Marveld1^tm1.1Liy Tg(GFAP-cre)25Mes/0	involves: C57BL/6J * FVB/N	MGI:6377096
cn52	Ren1^tm1.1Sig/Ren1^tm1.1Sig Tg(GFAP-cre)25Mes/0	involves: C57BL/6J * FVB/N	MGI:4939886
cn53	S1pr1^tm1Jch/S1pr1^tm1Jch Tg(GFAP-cre)25Mes/?	involves: C57BL/6J * FVB/N	MGI:4939155
cn54	Mldhr^tm1Zhgn/Mldhr^tm1Zhgn Tg(GFAP-cre)25Mes/0	involves: FVB/N	MGI:7612229
cn55	Fgfr1^tm1Upir/Fgfr1^tm1Upir Tg(GFAP-cre)25Mes/0	involves: FVB/N	MGI:3641103
cn56	Ntrk2^tm1Jom/Ntrk2^tm1Jom Tg(GFAP-cre)25Mes/0	involves: FVB/N	MGI:3612962
cn57	Lrp2^tm1Tew/Lrp2^tm1Tew Tg(GFAP-cre)25Mes/0	involves: FVB/N	MGI:6199477
cn58	Dio2^tm1Acb/Dio2^tm1Acb Tg(GFAP-cre)25Mes/0	involves: FVB/N	MGI:5513868
cn59	Bdnf^tm1Limm/Bdnf^tm1Limm Tg(GFAP-cre)25Mes/0	involves: FVB/N	MGI:3770149
cn60	Ric8a^tm1.1Zhua/Ric8a^tm1.1Zhua Tg(GFAP-cre)25Mes/0	involves: FVB/N	MGI:5467513

Genotype
MGI:5571382

cn1

• Allelic Composition: Hk2^tm1.1Uku/Hk2^tm1.1Uku; Tg(GFAP-cre)25Mes/0
• Genetic Background: B6.Cg-Hk2^tm1.1Uku Tg(GFAP-cre)25Mes

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

nervous system

abnormal cerebellar granule cell migration ( J:210113 )

• cerebellar granule neuron progenitors fail to migrate over the Purkinje cell layer

abnormal neuronal precursor proliferation ( J:210113 )

• at P7, cerebellar granule neuron progenitors exhibit reduced proliferation in response to Shh compared with wild-type cells

abnormal cerebellum external granule cell layer morphology ( J:210113 )

• focal disorganization of the external granule cell layer

• regions of focal thinning or thickening, abnormal migration and increased vascularization at P7

irregular external granule cell layer thickness ( J:210113 )

• regions of focal thinning or thickening at P7

cellular

abnormal cerebellar granule cell migration ( J:210113 )

• cerebellar granule neuron progenitors fail to migrate over the Purkinje cell layer

abnormal neuronal precursor proliferation ( J:210113 )

• at P7, cerebellar granule neuron progenitors exhibit reduced proliferation in response to Shh compared with wild-type cells

Genotype
MGI:4818692

cn2

• Allelic Composition: Mrtfa^tm1Eno/Mrtfa^tm1Eno; Mrtfb^tm2.1Eno/Mrtfb^tm2.1Eno; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129 * 129S/SvEv * C57BL/6 * FVB/N

mortality/aging

postnatal lethality, complete penetrance ( J:161853 )

• mice die between P16 and P21

nervous system

increased neuron apoptosis ( J:161853 )

• in the striatum

abnormal neuron differentiation ( J:161853 )

• neurons exhibit defective neurite outgrowth compared with wild-type neurons

abnormal neuronal migration ( J:161853 )

• neurons fail to migrate along the rostral migratory stream unlike in wild-type mice

absent corpus callosum ( J:161853 )

absent anterior commissure ( J:161853 )

decreased striatum size ( J:161853 )

• mice exhibit a smaller striatum than wild-type mice

abnormal hippocampus morphology ( J:161853 )

• the hippocampus is compressed unlike in wild-type mice

abnormal dentate gyrus morphology ( J:161853 )

abnormal hippocampus pyramidal cell layer ( J:161853 )

• poorly defined

abnormal hippocampus neuron morphology ( J:161853 )

• hippocampal neuronal projections are decreased compared to in wild-type mice

abnormal olfactory bulb morphology ( J:161853 )

• olfactory bulbs contain fewer neurons than in wild-type mice

abnormal rostral migratory stream morphology ( J:161853 )

• neurons fail to migrate along the rostral migratory stream unlike in wild-type mice

abnormal postnatal subventricular zone morphology ( J:161853 )

• mice exhibit an expansion of the subventricular zone compared with wild-type mice

• however, expansion is not do to increased cell proliferation

cellular

increased neuron apoptosis ( J:161853 )

• in the striatum

abnormal neuron differentiation ( J:161853 )

• neurons exhibit defective neurite outgrowth compared with wild-type neurons

impaired fibroblast cell migration ( J:161853 )

• mouse embryonic fibroblasts fail to migrate to the wound site in an in vitro scratch test unlike wild-type cells

abnormal neuronal migration ( J:161853 )

• neurons fail to migrate along the rostral migratory stream unlike in wild-type mice

growth/size/body

decreased body weight ( J:161853 )

• at P14

Genotype
MGI:5002697

cn3

• Allelic Composition: Pdcd10^tm1Wami/Pdcd10^tm1Wami; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129 * C57BL/6 * FVB/N

mortality/aging

premature death ( J:170480 )

• mice do not survive beyond 12 months of age

postnatal lethality, incomplete penetrance ( J:170480 )

• 80% of rate die by 4 weeks

nervous system

abnormal brain morphology ( J:170480 )

• abnormal cytoarchitecture

abnormal brain vasculature morphology ( J:170480 )

• brain capillaries are dilated and less organized and dense than in control mice

• between 3 weeks and 12 months, two-thirds of mice develop cerebrovascular lesions resembling cavernomas unlike in control mice

hydrocephaly ( J:170480 )

• at 2 months

increased brain size ( J:170480 )

dilated brain ventricle ( J:170480 )

astrocytosis ( J:170480 )

• starting in early postnatal stages

• astrocyte proliferation is increased compared to in control cells

• astrocytes exhibit resistance to cycloheximide-induced apoptosis compared with control cells

• retinal astrocyte spreading is decreased compared to in control mice

cardiovascular system

abnormal brain vasculature morphology ( J:170480 )

• brain capillaries are dilated and less organized and dense than in control mice

• between 3 weeks and 12 months, two-thirds of mice develop cerebrovascular lesions resembling cavernomas unlike in control mice

abnormal vascular endothelial cell migration ( J:170480 )

• endothelial cell migration is delayed compared to in control mice

dilated vasculature ( J:170480 )

• brain capillaries

neoplasm

increased hemangioma incidence ( J:170480 )

• between 3 weeks and 12 months, two-thirds of mice develop cerebrovascular lesions resembling cavernomas unlike in control mice

• mice develop cavernomas in the spinal cord unlike wild-type mice

behavior/neurological

abnormal gait ( J:170480 )

• unsteady

circling ( J:170480 )

growth/size/body

decreased body weight ( J:170480 )

postnatal growth retardation ( J:170480 )

cellular

abnormal vascular endothelial cell migration ( J:170480 )

• endothelial cell migration is delayed compared to in control mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
cerebral cavernous malformation 3		DOID:0060671	OMIM:603285	J:170480

Genotype
MGI:3613565

cn4

• Allelic Composition: Epor^tm1Hwu/Epor^tm1Hwu; Tg(GFAP-cre)25Mes/?
• Genetic Background: involves: 129 * C57BL/6 * FVB/N

nervous system

abnormal postnatal subventricular zone morphology ( J:104816 )

• subventricular zone decreased in volume and with fewer cells although the overall brain architecture of the cortex, olfactory bulbs and cerebellum is normal

• reduced cell proliferation at age 15 days with still greater reduction at 6 months of age

• infarct size from stroke not affected, contrary to expectations due to the neuroprotective effects of exogenous erythropoietin

Genotype
MGI:5472015

cn5

• Allelic Composition: Gt(ROSA)26Sor^{tm1(RICTOR)Jger}/Gt(ROSA)26Sor^{tm1(RICTOR)Jger}; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129 * FVB/N

Gliomas in Gt(ROSA)26Sor^{tm1(RICTOR)Jger}/Gt(ROSA)26Sor^{tm1(RICTOR)Jger} Tg(GFAP-cre)25Mes/0 mice

mortality/aging

premature death ( J:192206 )

• most mice succumb to gliomas by 22 weeks

neoplasm

abnormal tumor vascularization ( J:192206 )

• intratumoral hemorrhage

increased glioma incidence ( J:192206 )

• bilateral, multifocal infiltrating glioma in the amygdalohippocampal region or surrounding cortex in close proximity to the subventricular zone

increased oligodendroglioma incidence ( J:192206 )

• infiltrating gliomas exhibit oligodendroglial-like features with the typical fried egg appearance

nervous system

increased glioma incidence ( J:192206 )

• bilateral, multifocal infiltrating glioma in the amygdalohippocampal region or surrounding cortex in close proximity to the subventricular zone

increased oligodendroglioma incidence ( J:192206 )

• infiltrating gliomas exhibit oligodendroglial-like features with the typical fried egg appearance

abnormal postnatal subventricular zone morphology ( J:192206 )

• 2-fold increase in thickness

cardiovascular system

abnormal tumor vascularization ( J:192206 )

• intratumoral hemorrhage

Genotype
MGI:6358254

cn6

• Allelic Composition: Knl1^{tm1c(EUCOMM)Hmgu}/Knl1^{tm1c(EUCOMM)Hmgu}; Trp53^tm1Brn/Trp53^tm1Brn; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129P2/OlaHsd * 129S4/SvJaeSor * C57BL/6N * FVB/N
• Cell Lines: HEPD0665_2_E05

mortality/aging

postnatal lethality, complete penetrance ( J:278483 )

nervous system

nervous system phenotype ( J:278483 )

N • neuron loss is rescued by conditional knock-out of Trp53

telencephalon hypoplasia ( J:278483 )

• partially rescued by conditional knock-out of Trp53

cellular

aneuploidy ( J:278483 )

increased apoptosis ( J:278483 )

• partial rescue of apoptosis and DNA damage by conditional knock-out of Trp53

growth/size/body

microcephaly ( J:278483 )

• partially rescued by conditional knock-out of Trp53

Genotype
MGI:3831280

cn7

• Allelic Composition: Pten^tm1Rdp/Pten⁺; Trp53^tm1Brn/Trp53^tm1Brn; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129P2/OlaHsd * 129S6/SvEvTac * C57BL/6 * FVB/N

behavior/neurological

ataxia ( J:140704 )

• in 73% of mice at 15 to 40 weeks of age

paralysis ( J:140704 )

• in 73% of mice at 15 to 40 weeks of age

seizures ( J:140704 )

• in 73% of mice at 15 to 40 weeks of age

neoplasm

increased glioma incidence ( J:140704 )

• 73% of mice develop malignant gliomas consisting of anaplastic astrocytomas (in 66% of mice with tumors) and glioblastomas (in 34% of mice with tumors)

increased glioblastoma incidence ( J:140704 )

• 34% of mice with tumors develop glioblastomas

increased astrocytoma incidence ( J:140704 )

• anaplastic astrocytomas (in 66% of mice with tumors)

nervous system

seizures ( J:140704 )

• in 73% of mice at 15 to 40 weeks of age

increased glioma incidence ( J:140704 )

• 73% of mice develop malignant gliomas consisting of anaplastic astrocytomas (in 66% of mice with tumors) and glioblastomas (in 34% of mice with tumors)

increased glioblastoma incidence ( J:140704 )

• 34% of mice with tumors develop glioblastomas

increased astrocytoma incidence ( J:140704 )

• anaplastic astrocytomas (in 66% of mice with tumors)

Genotype
MGI:5571384

cn8

• Allelic Composition: Gt(ROSA)26Sor^{tm1(Smo/EYFP)Amc}/Gt(ROSA)26Sor⁺; Hk2^tm1.1Uku/Hk2⁺; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129P2/OlaHsd * 129X1/SvJ * C57BL/6 * FVB/N

mortality/aging

premature death ( J:210113 )

• mice die by P20

neoplasm

increased medulloblastoma incidence ( J:210113 )

nervous system

increased medulloblastoma incidence ( J:210113 )

Genotype
MGI:5571383

cn9

• Allelic Composition: Gt(ROSA)26Sor^{tm1(Smo/EYFP)Amc}/Gt(ROSA)26Sor⁺; Hk2^tm1.1Uku/Hk2^tm1.1Uku; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129P2/OlaHsd * 129X1/SvJ * C57BL/6 * FVB/N

mortality/aging

premature death ( J:210113 )

• median survival is 31 days compared with mice with wild-type Hk2 that die by P20

neoplasm

abnormal tumor vascularization ( J:210113 )

• increased micro-vascularization

decreased tumor growth/size ( J:210113 )

• compared to in mice with wild-type Hk2

increased medulloblastoma incidence ( J:210113 )

• all mice develop tumors with reduced growth and at increased latency compared to in mice with wild-type Hk2

increased tumor latency ( J:210113 )

• all mice develop tumors with increased latency compared to in mice with wild-type Hk2

nervous system

abnormal neuronal precursor proliferation ( J:210113 )

• reduced cerebellar granule neuron progenitors proliferation

increased medulloblastoma incidence ( J:210113 )

• all mice develop tumors with reduced growth and at increased latency compared to in mice with wild-type Hk2

cellular

abnormal neuronal precursor proliferation ( J:210113 )

• reduced cerebellar granule neuron progenitors proliferation

cardiovascular system

abnormal tumor vascularization ( J:210113 )

• increased micro-vascularization

Genotype
MGI:3804817

cn10

• Allelic Composition: Mib1^tm2Kong/Mib1^tm2Kong; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129P2/OlaHsd * C57BL/6 * FVB/N

mortality/aging

premature death ( J:130369 )

• mice die at 2 to 4 months of age

nervous system

abnormal neuronal migration ( J:130369 )

• mice exhibit a delay in the migration of cells from the external granule layer to the internal granule layer

reduced cerebellar foliation ( J:130369 )

• reduced at P9

abnormal cerebellum external granule cell layer morphology ( J:130369 )

• at P9, the external granule cell layer contains more granule cells than in wild-type mice

• unlike in wild-type mice, granule cells are maintained at P17 but migrate to the internal granule cell layer by P21

• however, cell proliferation within the external granule layer is normal

small cerebellum ( J:130369 )

• at P9

abnormal CNS glial cell morphology ( J:130369 )

• at P15, Bergmann glial cell processes are severely disorganized

growth/size/body

decreased body size ( J:130369 )

cellular

abnormal neuronal migration ( J:130369 )

• mice exhibit a delay in the migration of cells from the external granule layer to the internal granule layer

Genotype
MGI:3831281

cn11

• Allelic Composition: Trp53^tm1Brn/Trp53^tm1Brn; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129P2/OlaHsd * C57BL/6 * FVB/N

neoplasm

increased astrocytoma incidence ( J:140704 )

• 4 of 23 mice develop anaplastic astrocytomas

nervous system

increased astrocytoma incidence ( J:140704 )

• 4 of 23 mice develop anaplastic astrocytomas

Genotype
MGI:4462797

cn12

• Allelic Composition: Glul^tm3Whla/Glul^tm3Whla; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129P2/OlaHsd * FVB/N

mortality/aging

postnatal lethality, complete penetrance ( J:162067 )

• born in normal numbers

• none found at 6-8 days of age

• death at 2-3 days of age

behavior/neurological

abnormal food intake ( J:162067 )

• nursed immediately after birth but milk intake then declined

homeostasis/metabolism

abnormal amino acid level ( J:162067 )

• decreased glutamine and alanine in the cortex of the brain at two days of age

decreased glutamine level ( J:162067 )

• decreased glutamine in the cortex of the brain at two days of age

increased glycine level ( J:162067 )

• increased glycine in the cortex of the brain at two days of age

abnormal ammonia homeostasis ( J:162067 )

• moderately elevated brain ammonia levels

decreased circulating glucose level ( J:162067 )

• blood glucose levels at 30% of controls at 2 days of age

nervous system

abnormal brain morphology ( J:162067 )

• moderately elevated brain ammonia levels

• no gross brain malformations

Genotype
MGI:4881761

cn13

• Allelic Composition: Phgdh^tm1.2Shfu/Phgdh^tm1.2Shfu; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129P2/OlaHsd * FVB/N

nervous system

decreased brain weight ( J:167572 )

• at 6 weeks but not at P0

forebrain hypoplasia ( J:167572 )

homeostasis/metabolism

decreased arginine level ( J:167572 )

• arginine levels in the cerebral cortex are decreased compared to in control mice

increased arginine level ( J:167572 )

• arginine levels in the hippocampus are increased compared to in control mice

increased circulating glycine level ( J:167572 )

• serum glycine concentration is subtly increased compared to in control mice

decreased cystathionine level ( J:167572 )

• cystathionine levels in the cerebral cortex and hippocampus are decreased compared to in control mice

decreased glutamic acid level ( J:167572 )

• glutamic acid levels in the hippocampus are decreased compared to in control mice

increased glutamine level ( J:167572 )

• glutamine levels in the hippocampus are increased compared to in control mice

decreased glycine level ( J:167572 )

• free glycine levels in the cerebral cortex are decreased compared to in control mice

decreased phenylalanine level ( J:167572 )

• phenylalanine levels in the cerebral cortex are decreased compared to in control mice

increased phenylalanine level ( J:167572 )

• phenylalanine levels in the hippocampus are increased compared to in control mice

decreased serine level ( J:167572 )

• free L- and D-serine levels in the cerebral cortex and hippocampus are decreased compared to in control mice

• L-serine or D-serine injection results in a greater increase in L-serine and D-serine content than in similarly treated control mice

decreased threonine level ( J:167572 )

• threonine levels in the hippocampus are decreased compared to in control mice

growth/size/body

microcephaly ( J:167572 )

• at P42 but not at P0

Genotype
MGI:5495325

cn14

• Allelic Composition: Cdc42^tm1.1Rac/Cdc42^tm1.1Rac; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129P2/OlaHsd * FVB/N

mortality/aging

premature death ( J:197886 )

• more than half of mice die before 3 months of age due to severe hydrocephalus

nervous system

hydrocephaly ( J:197886 )

• severe

abnormal brain ventricle morphology ( J:197886 )

• ependymal cells are detached from the lateral and third ventricle

abnormal brain ependyma morphology ( J:197886 )

• ependymal cells are detached from the lateral and third ventricle

absent brain ependyma motile cilia ( J:197886 )

enlarged lateral ventricles ( J:197886 )

enlarged third ventricle ( J:197886 )

thin cerebral cortex ( J:197886 )

craniofacial

domed cranium ( J:197886 )

• in most mice at 4 weeks

skeleton

domed cranium ( J:197886 )

• in most mice at 4 weeks

cellular

absent brain ependyma motile cilia ( J:197886 )

Genotype
MGI:4462796

cn15

• Allelic Composition: Glul^tm3Whla/Glul^tm1Whla; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129P2/OlaHsd * FVB/N

mortality/aging

postnatal lethality, complete penetrance ( J:162067 )

• born in normal numbers

• none found at 6-8 days of age

• death at 2-3 days of age

behavior/neurological

abnormal food intake ( J:162067 )

• nursed immediately after birth but milk intake then declined

nervous system

abnormal brain morphology ( J:162067 )

• moderately elevated brain ammonia levels

• no gross brain malformations

homeostasis/metabolism

abnormal amino acid level ( J:162067 )

• decreased alanine in the cortex of the brain at two days of age

decreased glutamine level ( J:162067 )

• decreased glutamine in the cortex of the brain at two days of age

increased glycine level ( J:162067 )

• increased glycine in the cortex of the brain at two days of age

abnormal ammonia homeostasis ( J:162067 )

• moderately elevated brain ammonia levels

decreased circulating glucose level ( J:162067 )

• blood glucose levels at 30% of controls at 2 days of age

Genotype
MGI:4840094

cn16

• Allelic Composition: Nf1^tm1Par/Nf1⁺; Pten^tm1Hwu/Pten⁺; Trp53^tm1Tyj/Trp53⁺; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129S1/Sv * 129S2/SvPas * 129S4/SvJae * 129X1/SvJ * FVB/N

mortality/aging

premature death ( J:134611 )

• most die as early as 10 weeks of age, and usually within a week of showing signs of morbidity

behavior/neurological

increased startle reflex ( J:134611 )

• exaggerated startle response

ataxia ( J:134611 )

• ataxic gait

unidirectional circling ( J:134611 )

seizures ( J:134611 )

• generalized motor seizures

nervous system

seizures ( J:134611 )

• generalized motor seizures

increased brain tumor incidence ( J:134611 )

increased astrocytoma incidence ( J:134611 )

• gliomas from asymptomatic mutants are primarily classified as grade 3 astocytomas

• 5-fold higher growth rate of astrocytomas compared with tumors from transgenic mice heterozygous for Nf1^tm1Par and Trp53^tm1Tyj and hemizygous for Tg(GFAP-cre)25Mes

neoplasm

increased brain tumor incidence ( J:134611 )

increased astrocytoma incidence ( J:134611 )

• gliomas from asymptomatic mutants are primarily classified as grade 3 astocytomas

• 5-fold higher growth rate of astrocytomas compared with tumors from transgenic mice heterozygous for Nf1^tm1Par and Trp53^tm1Tyj and hemizygous for Tg(GFAP-cre)25Mes

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
malignant astrocytoma		DOID:3069		J:134611

Genotype
MGI:4840090

cn17

• Allelic Composition: Nf1^tm1Par/Nf1⁺; Trp53^tm1Tyj/Trp53⁺; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129S1/Sv * 129S2/SvPas * 129X1/SvJ * FVB/N

mortality/aging

premature death ( J:134611 )

• mutants survive up to 8 weeks beyond initial appearance of symptoms

neoplasm

increased brain tumor incidence ( J:134611 )

increased astrocytoma incidence ( J:134611 )

• complete penetrance of malignant astrocytomas

• gliomas from asymptomatic mutants are primarily classified as grade 2 astocytomas

• neurospheres from mutant astrocytomas exhibit loss of heterozygosity at both Nf1 and Trp53 loci

nervous system

increased brain tumor incidence ( J:134611 )

increased astrocytoma incidence ( J:134611 )

• complete penetrance of malignant astrocytomas

• gliomas from asymptomatic mutants are primarily classified as grade 2 astocytomas

• neurospheres from mutant astrocytomas exhibit loss of heterozygosity at both Nf1 and Trp53 loci

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
malignant astrocytoma		DOID:3069		J:134611

Genotype
MGI:4840096

cn18

• Allelic Composition: Nf1^tm1Par/Nf1⁺; Pten^tm1Hwu/Pten⁺; Trp53^tm1Elee/Trp53⁺; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129S1/Sv * 129S4/SvJae * 129X1/SvJ * FVB/N

mortality/aging

premature death ( J:134611 )

• most die as early as 10 weeks of age, and usually within a week of showing signs of morbidity

behavior/neurological

increased startle reflex ( J:134611 )

• exaggerated startle response

ataxia ( J:134611 )

• ataxic gait

unidirectional circling ( J:134611 )

seizures ( J:134611 )

• generalized motor seizures

nervous system

seizures ( J:134611 )

• generalized motor seizures

increased brain tumor incidence ( J:134611 )

increased astrocytoma incidence ( J:134611 )

• mutants develop astrocytomas

• neurospheres from mutant astrocytomas exhibit loss of heterozygosity at both Nf1 and Trp53 loci

neoplasm

increased brain tumor incidence ( J:134611 )

increased astrocytoma incidence ( J:134611 )

• mutants develop astrocytomas

• neurospheres from mutant astrocytomas exhibit loss of heterozygosity at both Nf1 and Trp53 loci

Genotype
MGI:4840095

cn19

• Allelic Composition: Nf1^tm1Par/Nf1⁺; Trp53^tm1Elee/Trp53⁺; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129S1/Sv * 129S4/SvJae * 129X1/SvJ * FVB/N

mortality/aging

premature death ( J:134611 )

• mutants survive up to 8 weeks beyond initial appearance of symptoms

behavior/neurological

increased startle reflex ( J:134611 )

• exaggerated startle response

ataxia ( J:134611 )

• ataxic gait

unidirectional circling ( J:134611 )

seizures ( J:134611 )

• generalized motor seizures

nervous system

seizures ( J:134611 )

• generalized motor seizures

increased brain tumor incidence ( J:134611 )

increased astrocytoma incidence ( J:134611 )

• mutants develop astrocytomas

• neurospheres from mutant astrocytomas exhibit loss of heterozygosity at both Nf1 and Trp53 loci

neoplasm

increased brain tumor incidence ( J:134611 )

increased astrocytoma incidence ( J:134611 )

• mutants develop astrocytomas

• neurospheres from mutant astrocytomas exhibit loss of heterozygosity at both Nf1 and Trp53 loci

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
malignant astrocytoma		DOID:3069		J:134611

Genotype
MGI:3805515

cn20

• Allelic Composition: Mapk1^tm1Gela/Mapk1^tm1Gela; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ * FVB/N

behavior/neurological

abnormal contextual conditioning behavior ( J:137882 )

• long-term memory for contextual conditioning is significantly impaired compared to wild-type controls

• overtraining does not rescue defect as mice continue to display less freezing behavior than wild-type littermates

abnormal cued conditioning behavior ( J:137882 )

• long-term memory for cued conditioning is significantly impaired compared to wild-type controls

• overtraining does not rescue defect as mice continue to display less freezing behavior than wild-type littermates

nervous system

abnormal neuron differentiation ( J:137882 )

abnormal cerebral cortex morphology ( J:137882 )

• cellular density appears greater throughout cortex, but laminar organization is normal

• at P10 mice show a 10% reduction in neuron number in cerebral cortex, with a 40% increase in non-neuronal, presumptive glial cells

• neuron number in layer VI and cortical preplate is reduced by 35%at P2

• in layer V, there are only 50% as many neurons as in wild-type at P2

• layers II-IV show a 30% decrease in neurons at P2

• dramatic increase in astrocyte number is observed throughout cortex and subventricular zone

• astrocyte metabolism and viability are similar to wild-type; neuronal apoptosis and differentiation are similar to controls

thin cerebral cortex ( J:137882 )

• cortical thickness is significantly reduced throughout the dorsal telencephalon relative to wild-type at P2

cellular

abnormal neuron differentiation ( J:137882 )

Genotype
MGI:2684282

cn21

• Allelic Composition: Dag1^tm1Kcam/Dag1^tm2Kcam; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ * FVB/N

nervous system

abnormal neuronal migration ( J:86901 )

• cerebral, cerebellar and brain stem neuronal migration abnormalities

increased brain size ( J:86901 )

cellular

abnormal neuronal migration ( J:86901 )

• cerebral, cerebellar and brain stem neuronal migration abnormalities

growth/size/body

megacephaly ( J:86901 )

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
lissencephaly		DOID:0050453	OMIM:PS607432	J:86901

Genotype
MGI:2684281

cn22

• Allelic Composition: Dag1^tm2Kcam/Dag1^tm2Kcam; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ * FVB/N

nervous system

abnormal neuronal migration ( J:86901 )

• cerebral, cerebellar and brain stem neuronal migration abnormalities

abnormal brain morphology ( J:86901 )

• fusion of the cerebral interhemispheric fissure and adjacent cerebellar folia

• malformations resembling polymicrogyria

abnormal cerebellum development ( J:86901 )

• granule cells are observed in the subrarachnoid space during postnatal cerebellar development indicating aberrant migration of granule cells

increased brain size ( J:86901 )

• about 20% increase in brain size

abnormal hippocampus CA1 region morphology ( J:86901 )

• some mutants show minor dispersion of neuronal cell bodies in the CA1 region

abnormal hippocampus granule cell layer ( J:86901 )

• some mutants show focal irregularities of the dentate granule cell layer in the hippocampus

abnormal cerebral cortex morphology ( J:86901 )

• cerebral cortical surfaces show widespread discontinuities of the glia limitans (pial surface basal lamina) accompanied by glial neuronal heterotopia within the leptomeninges

abnormal stratification in cerebral cortex ( J:86901 )

• multifocal disarray of neuronal layering in the cerebral cortex

abnormal cerebellar cortex morphology ( J:86901 )

• cerebellar cortical surfaces show widespread discontinuities of the glia limitans (pial surface basal lamina) accompanied by glial neuronal heterotopia within the leptomeninges

astrocytosis ( J:86901 )

• GFAP-immunoreactive astrocytes are prominent in the cerebral cortex, indicating gliosis

abnormal subarachnoid space morphology ( J:86901 )

• the normally open subarachnoid space is filled with heterotopic astrocytic and neuronal processes

reduced long-term potentiation ( J:86901 )

• induction of long-term potentiation (LTP) by high-frequency stimulation is blunted in hippocampal slices, however baseline neurotransmission is unaffected and presynaptic neurotransmitter release is not affected

vision/eye

abnormal rod electrophysiology ( J:158199 )

• significantly reduced positive scotopic threshold response

• significantly increased negative scotopic threshold response

• the b-wave responses are severely attenuated

cellular

abnormal neuronal migration ( J:86901 )

• cerebral, cerebellar and brain stem neuronal migration abnormalities

growth/size/body

megacephaly ( J:86901 )

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
lissencephaly		DOID:0050453	OMIM:PS607432	J:86901

Genotype
MGI:5472016

cn23

• Allelic Composition: Gt(ROSA)26Sor^{tm1(RICTOR)Jger}/Gt(ROSA)26Sor^{tm1(RICTOR)Jger}; Tg(GFAP-cre)25Mes/0; Tg(GFAP-EGFR*,-lacZ)#Agu/0
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ * FVB/N

Increased grade of oligodendroglioma in Gt(ROSA)26Sor^{tm1(RICTOR)Jger}/Gt(ROSA)26Sor^{tm1(RICTOR)Jger} Tg(GFAP-EGFR*,-lacZ)#Agu/0 Tg(GFAP-cre)25Mes/0 mice

mortality/aging

premature death ( J:192206 )

• all mice succumb to gliomas by 18 weeks

neoplasm

increased glioma incidence ( J:192206 )

• mice exhibit accelerated glioma development compared with Gt(ROSA)26Sor^{tm1(RICTOR)Jger}/Gt(ROSA)26Sor^{tm1(RICTOR)Jger} Tg(GFAP-cre)25Mes mice

increased astrocytoma incidence ( J:192206 )

• mixed astrocytic-oligodendroglial tumors

increased oligodendroglioma incidence ( J:192206 )

• mixed astrocytic-oligodendroglial tumors

decreased tumor latency ( J:192206 )

• mice exhibit accelerated glioma development compared with Gt(ROSA)26Sor^{tm1(RICTOR)Jger}/Gt(ROSA)26Sor^{tm1(RICTOR)Jger} Tg(GFAP-cre)25Mes mice

nervous system

increased glioma incidence ( J:192206 )

• mice exhibit accelerated glioma development compared with Gt(ROSA)26Sor^{tm1(RICTOR)Jger}/Gt(ROSA)26Sor^{tm1(RICTOR)Jger} Tg(GFAP-cre)25Mes mice

increased astrocytoma incidence ( J:192206 )

• mixed astrocytic-oligodendroglial tumors

increased oligodendroglioma incidence ( J:192206 )

• mixed astrocytic-oligodendroglial tumors

abnormal postnatal subventricular zone morphology ( J:192206 )

• 3-fold increase in thickness

Genotype
MGI:4436771

cn24

• Allelic Composition: Ntf3^tm1Esm/Ntf3^tm1Esm; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129S1/Sv * FVB/N

nervous system

abnormal nervous system tract morphology ( J:81563 )

• loss of subcortical tract results in a reduction in axonal bundles in subcortical white matter

vision/eye

abnormal vision ( J:81563 )

• impaired visual function indicated by the visual cliff avoidance test: mutants often cross the cliff edge without hesitation and show no preference for either side of the box

Genotype
MGI:3849178

cn25

• Allelic Composition: Trp53^tm1Elee/Trp53^tm1Tyj; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129S2/SvPas * 129S4/SvJae * FVB/N

mortality/aging

premature death ( J:149662 )

• all mice die prior to 500 days

behavior/neurological

tremors ( J:149662 )

• 85% of mice

ataxia ( J:149662 )

• 85% of mice

impaired balance ( J:149662 )

• 85% of mice

seizures ( J:149662 )

• 85% of mice

neoplasm

increased medulloblastoma incidence ( J:149662 )

• 90% of mice develop medulloblastomas

increased sarcoma incidence ( J:149662 )

• mice develop tumors outside of the central nervous system consisting of soft-tissue sarcomas

increased glioma incidence ( J:149662 )

• 90% of mice develop malignant gliomas with astrocytic characteristics

• 40% of high-grade astrocytic gliomas exhibit necrosis, pseudopalisading tumors cells, high degree of nuclear atypia, and microvascular proliferation similar to human glioblastoma multiforme

• tumors are relatively heterogeneous histological and in lineage marker expression

nervous system

nervous system phenotype ( J:149662 )

N • at 2 months of age, brain cells in the subventricular zone and progenitor cells exhibit normal growth rates

seizures ( J:149662 )

• 85% of mice

increased medulloblastoma incidence ( J:149662 )

• 90% of mice develop medulloblastomas

increased glioma incidence ( J:149662 )

• 90% of mice develop malignant gliomas with astrocytic characteristics

• 40% of high-grade astrocytic gliomas exhibit necrosis, pseudopalisading tumors cells, high degree of nuclear atypia, and microvascular proliferation similar to human glioblastoma multiforme

• tumors are relatively heterogeneous histological and in lineage marker expression

increased brain size ( J:149662 )

• in mice with neurological defects

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
glioblastoma		DOID:3068		J:149662

Genotype
MGI:3839880

cn26

• Allelic Composition: Kmt2a^tm1.1Erns/Kmt2a^tm1.1Erns; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129S2/SvPas * FVB/N

mortality/aging

premature death ( J:147104 )

• mice die between P25 and P30

nervous system

abnormal neuron differentiation ( J:147104 )

• cultured subventricular cells produce fewer neuronal cells while remaining gliogenic unlike wild-type cells

• however, cultured subventricular cells exhibit normal proliferation and apoptosis

abnormal neuronal precursor cell migration ( J:147104 )

• neuroblast chain migration in mice and from subventricular zone explants is severely disorganized, and migration is reduced 40% compared to wild-type cells

abnormal brain development ( J:147104 )

• all regions of the brain that undergo postnatal neurogenesis including the cerebellar granule cell layer, hippocampal dentate gyrus, and olfactory bulb are reduced in size compared to in wild-type mice

• however, the ependymal layer and oligodendrocytes develop normally

abnormal dentate gyrus morphology ( J:147104 )

• reduced in size with reduced neurons

small olfactory bulb ( J:147104 )

• with reduced neurons

thin cerebellar granule layer ( J:147104 )

• with reduced neurons

abnormal postnatal subventricular zone morphology ( J:147104 )

• the expanded subventricular zone contains 3- to 4-fold fewer proliferating neuroblasts compared to in wild-type mice

• after P7, neuroblasts accumulate in the subventricular zone due to impaired migration unlike in wild-type mice

• the subventricular zone contains more cells positive for an astrocyte-specific marker than in wild-type mice

abnormal astrocyte morphology ( J:147104 )

• the subventricular zone and other brain regions contains more cells positive for an astrocyte-specific marker than in wild-type mice

decreased neuronal precursor cell number ( J:147104 )

• the subventricular zone contains 3- to 4-fold fewer proliferating neuroblasts compared to in wild-type mice

• under differentiation condition, cultured subventricular zone cells produce more than 20-fold fewer Tuj1+ (marking neuron precursor cells) compared similarly treated wild-type cells

behavior/neurological

ataxia ( J:147104 )

growth/size/body

postnatal growth retardation ( J:147104 )

• progressive

cellular

abnormal neuron differentiation ( J:147104 )

• cultured subventricular cells produce fewer neuronal cells while remaining gliogenic unlike wild-type cells

• however, cultured subventricular cells exhibit normal proliferation and apoptosis

abnormal neuronal precursor cell migration ( J:147104 )

• neuroblast chain migration in mice and from subventricular zone explants is severely disorganized, and migration is reduced 40% compared to wild-type cells

Genotype
MGI:3615302

cn27

• Allelic Composition: Olig2^tm1Qrlu/Olig2^tm1Qrlu; Tg(GFAP-cre)25Mes/?
• Genetic Background: involves: 129S4/SvJae * C57BL/6J * FVB/N

behavior/neurological

limb grasping ( J:105071 )

• abnormal limb clasping reflex

• beginning at 2 weeks of age

tremors ( J:105071 )

• beginning at 2 weeks of age

nervous system

abnormal corpus callosum morphology ( J:105071 )

• severe reduction in numbers of myelinated axons and myelin sheaths are thinner

abnormal oligodendrocyte morphology ( J:105071 )

• development arrested in dorsal cortex before formation of cells capable of forming myelin

Genotype
MGI:3849177

cn28

• Allelic Composition: Trp53^tm1Elee/Trp53^tm1Elee; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129S4/SvJae * FVB/N

mortality/aging

premature death ( J:149662 )

• all mice die prior to 400 days

behavior/neurological

tremors ( J:149662 )

• 84% of mice

ataxia ( J:149662 )

• 84% of mice

impaired balance ( J:149662 )

• 84% of mice

seizures ( J:149662 )

• 84% of mice

neoplasm

increased medulloblastoma incidence ( J:149662 )

• 90% of mice develop medulloblastomas

increased sarcoma incidence ( J:149662 )

• mice develop tumors outside of the central nervous system consisting of soft-tissue sarcomas

increased glioma incidence ( J:149662 )

• 90% of mice develop malignant gliomas with astrocytic characteristics

• 40% of high-grade astrocytic gliomas exhibit necrosis, pseudopalisading tumors cells, high degree of nuclear atypia, and microvascular proliferation similar to human glioblastoma multiforme

• tumors are relatively heterogeneous histological and in lineage marker expression

nervous system

nervous system phenotype ( J:149662 )

N • at 2 months of age, brain cells in the subventricular zone and progenitor cells exhibit normal growth rates

seizures ( J:149662 )

• 84% of mice

increased medulloblastoma incidence ( J:149662 )

• 90% of mice develop medulloblastomas

increased glioma incidence ( J:149662 )

• 90% of mice develop malignant gliomas with astrocytic characteristics

• 40% of high-grade astrocytic gliomas exhibit necrosis, pseudopalisading tumors cells, high degree of nuclear atypia, and microvascular proliferation similar to human glioblastoma multiforme

• tumors are relatively heterogeneous histological and in lineage marker expression

increased brain size ( J:149662 )

• in mice with neurological defects

Genotype
MGI:4830362

cn29

• Allelic Composition: Pten^tm1Hwu/Pten^tm1Hwu; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129S4/SvJae * FVB/N

mortality/aging

lethality at weaning, complete penetrance ( J:100428 )

• death occurs around P21

nervous system

abnormal cerebellum development ( J:100428 )

• cerebella layering defects

• before P3, cerebella are enlarged but the external granule layer (EGL) and cerebellar lobules are normal and Purkinje cell position is normal

• however, after P6, mutants show a marked cerebellar enlargement and lack internal granule layer and folia

abnormal cerebellar foliation ( J:100428 )

• loss of foliation after P6

increased brain size ( J:100428 )

abnormal cerebellar Purkinje cell layer ( J:100428 )

• Purkinje layer defect is first seen at P4-P6 and by P9, numerous Purkinje neurons are randomly scattered

abnormal Bergmann glial cell morphology ( J:100428 )

• disruption of Bergmann glial layering

abnormal Bergmann glial cell differentiation ( J:100428 )

• premature differentiation of Bergmann glia leading to extensive layering defects

ectopic Bergmann glia cells ( J:100428 )

• some Bergmann cell bodies are randomly distributed at P7 and lose their contacts to the pial surface and are positioned deep within the internal granule layer region

ectopic Purkinje cell ( J:100428 )

• Purkinje cell layer is normal at P3 but there are many ectopic Purkinje cells by P9

abnormal cerebellar granule layer morphology ( J:100428 )

• lack of an organized internal granule layer (IGL) at P6

• severe granule neuron layering defects

abnormal cerebellar granule cell morphology ( J:100428 )

• mutants exhibit granule neuron migration defects, with granule neurons failing to migrate to the internal granule layer and accumulating in the molecular layer (ML)

• granule neurons are resistant to low potassium-induced cell death under serum deprivation conditions

enlarged cerebellum ( J:100428 )

cellular

abnormal Bergmann glial cell differentiation ( J:100428 )

• premature differentiation of Bergmann glia leading to extensive layering defects

growth/size/body

megacephaly ( J:100428 )

Genotype
MGI:5302861

cn30

• Allelic Composition: Pomt2^tm1.1Hhu/Pomt2^tm1.1Hhu; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129S4/SvJaeSor * 129S6/SvEvTac * C57BL/6J * FVB/N

nervous system

abnormal cerebellar granule cell migration ( J:179356 )

• some granule cells fail to migrate in the cerebellum

• however, forebrain architecture is normal

abnormal cerebellum morphology ( J:179356 )

• adult mice exhibit breaches in the pial basement membrane and the glia limitans in limited areas at the midline between the cerebral hemispheres compared to in wild-type mice

cellular

abnormal cerebellar granule cell migration ( J:179356 )

• some granule cells fail to migrate in the cerebellum

• however, forebrain architecture is normal

abnormal basement membrane morphology ( J:179356 )

• adult mice exhibit breaches in the pial basement membrane and the glia limitans in limited areas at the midline between the cerebral hemispheres compared to in wild-type mice

Genotype
MGI:6358252

cn31

• Allelic Composition: Knl1^{tm1c(EUCOMM)Hmgu}/Knl1^{tm1c(EUCOMM)Hmgu}; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129S4/SvJaeSor * C57BL/6N * FVB/N
• Cell Lines: HEPD0665_2_E05

mortality/aging

decreased survivor rate ( J:278483 )

postnatal lethality, incomplete penetrance ( J:278483 )

nervous system

premature neuronal precursor differentiation ( J:278483 )

• neural progenitor cells prematurely exhibit the cell cycle and become migratory

microgliosis ( J:278483 )

• mis-segregated chromosomes with DNA bridges located at the cleavage furrow

• however, karyotypes are normal

thin cortical plate ( J:278483 )

absent corpus callosum ( J:278483 )

telencephalon hypoplasia ( J:278483 )

decreased neuronal precursor cell number ( J:278483 )

• at E15.5 and E16.5 with some precursor cells located outside the ventricular zone and subventricular zone

decreased neuron number ( J:278483 )

• due to increased apoptosis

• however, deep layer neuron numbers are normal

• POU3F2-expressing L2-5 neurons

• CUX1-expressing L2-4 neurons

cellular

abnormal mitotic cytokinesis ( J:278483 )

• mis-segregated chromosomes with DNA bridges located at the cleavage furrow

• however, karyotypes are normal

increased apoptosis ( J:278483 )

• in the cortex at E15.5, abundant in the germinal zones and intermediate zone

• marginally by E17.5

• associated with increased DNA damage determined by gammaH2AX staining

• however, apoptotic cells are largely absent from the cortical plate

premature neuronal precursor differentiation ( J:278483 )

• neural progenitor cells prematurely exhibit the cell cycle and become migratory

abnormal double-strand DNA break repair ( J:278483 )

• increased DSB repair

growth/size/body

microcephaly ( J:278483 )

• less severe than in mice with the conditional activated by Emx1^tm1(cre)Krj

decreased body size ( J:278483 )

homeostasis/metabolism

abnormal double-strand DNA break repair ( J:278483 )

• increased DSB repair

immune system

microgliosis ( J:278483 )

• mis-segregated chromosomes with DNA bridges located at the cleavage furrow

• however, karyotypes are normal

hematopoietic system

microgliosis ( J:278483 )

• mis-segregated chromosomes with DNA bridges located at the cleavage furrow

• however, karyotypes are normal

Genotype
MGI:6449671

cn32

• Allelic Composition: Cul3^tm1Jdsr/Cul3⁺; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129S4/SvJaeSor * FVB/N

behavior/neurological

behavior/neurological phenotype ( J:292173 )

N • normal latency to fall in rotarod test

N • normal distance traveled in open field test

N • normal number of arm entries and spontaneous alterations in Y-maze test

N • normal time and number of grooming episodes

increased anxiety-related response ( J:292173 )

• fewer entries into and time spent in open arms in elevated plus maze test

• normal total distance travelled and time spent in closed arms in elevated plus maze test

increased thigmotaxis ( J:292173 )

• less time spent in center in open field test

• normal distance traveled in open field test

abnormal response to social novelty ( J:292173 )

• reduced social preference index when introduced to novel mouse and novel inanimate object in three-chamber test

• reduced social preference index when introduced to novel mouse and known mouse in three-chamber test

nervous system

nervous system phenotype ( J:292173 )

N • normal cerebral cortex, corpus callosum and hippocampus morphology and brain weight

N • normal latency to localize buried food pellets in olfactory sensing test

N • normal neuron morphology in CA1 and CA3 hippocampal regions and in cerebral neocortex layers at age P60

N • normal length and complexity of apical and basal dendrites of hippocampal CA1 neurons at age P60

N • normal resting membrane potential (RMP) in hippocampal CA1 pyramidal neurons at age P60

N • normal number of inhibitory synapses in hippocampal CA1 pyramidal neurons at age P15

increased dendritic spine density ( J:292173 )

• increased number of spines on apical dendrites of hippocampal CA1 neurons at age P15 and P60

abnormal action potential ( J:292173 )

• increased AP frequency in response to injected currents in hippocampal CA1 pyramidal neurons at age P60

abnormal CNS synaptic transmission ( J:292173 )

• higher rate of successful responses and synaptic efficacy to minimal stimulation in hippocampal CA1 pyramidal neurons at age P60

• increased EPSC/IPSC ratio in hippocampal CA1 pyramidal neurons at age P60

• normal synaptic potency to minimal stimulation in hippocampal CA1 pyramidal neurons at age P60

increased miniature excitatory postsynaptic current frequency ( J:292173 )

• increased mEPSC frequency in hippocampal CA1 pyramidal neurons at age P15 and P60

• normal mEPSC amplitude in hippocampal CA1 pyramidal neurons at age P15 and P60

increased miniature inhibitory postsynaptic current frequency ( J:292173 )

• increased mIPSC frequency in hippocampal CA1 pyramidal neurons at age P60

• normal mIPSC amplitude in hippocampal CA1 pyramidal neurons at age P60

decreased paired-pulse facilitation ( J:292173 )

• reduced paired-pulse ratio in hippocampal CA1 pyramidal neurons at age P60

mortality/aging

mortality/aging ( J:292173 )

N • viable and normal lifespan

growth/size/body

growth/size/body region phenotype ( J:292173 )

N • normal body size

reproductive system

reproductive system phenotype ( J:292173 )

N • fertile

Genotype
MGI:6449669

cn33

• Allelic Composition: Cul3^tm1Jdsr/Cul3^tm1Jdsr; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129S4/SvJaeSor * FVB/N

nervous system

decreased brain weight ( J:292173 )

absent corpus callosum ( J:292173 )

abnormal hippocampus morphology ( J:292173 )

• deformed hippocampus

abnormal stratification in cerebral cortex ( J:292173 )

thin cerebral cortex ( J:292173 )

mortality/aging

preweaning lethality ( J:292173 )

• mice die before age P17

growth/size/body

decreased body size ( J:292173 )

Genotype
MGI:5896534

cn34

• Allelic Composition: Nsdhl^tm1.1Hrm/Y; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129S6/SvEvTac * C57BL/6J * FVB/N

mortality/aging

premature death ( J:220990 )

♂ • die between P12-P20

behavior/neurological

ataxia ( J:220990 )

♂ • by P8 to P10

nervous system

thin external granule cell layer ( J:220990 )

♂ • thinner with fewer granule cell precursors at P5 and becoming progressively worse with age

♂ • at P8 only a single layer of granule cell precursors is present in severely affected mice

abnormal hippocampus morphology ( J:220990 )

♂ • appears disorganized with fewer hippocampal neurons

abnormal dentate gyrus morphology ( J:220990 )

♂ • fewer neurons present in the dentate gyrus at P0

abnormal hippocampus neuron morphology ( J:220990 )

♂ • fewer hippocampal neurons at P0 especially in the dentate gyrus

abnormal hippocampus granule cell morphology ( J:220990 )

♂ • reduced numbers and organization of granule neurons at P7

small hippocampus ( J:220990 )

♂ • at P7

abnormal cerebral cortex morphology ( J:220990 )

♂ • at P7

♂ • increase in pycnotic nuclei in the outer layer

abnormal Bergmann glial cell morphology ( J:220990 )

♂ • fewer, shorter cells that appear disorganized

abnormal cerebellar granule cell morphology ( J:220990 )

♂ • reduced numbers and organization of granule neurons at P7

♂ • decreased cholesterol levels and increased levels of sterol intermediates in granule cell precursors at P3, P5, and P7

decreased cerebellar granule cell number ( J:220990 )

♂ • at P7

small cerebellum ( J:220990 )

♂ • at P7

abnormal neurite morphology ( J:220990 )

♂ • cultured granule cell precursors produce fewer and thinner neurite extensions

♂ • cell morphology is improved with the addition of cholesterol to the culture medium

abnormal Purkinje cell dendrite morphology ( J:220990 )

♂ • cerebellar Purkinje cells fail to form extensive dendritic trees unlike wild-type controls

♂ • dendritic trees eventually degenerate

abnormal neuronal stem cell physiology ( J:220990 )

♂ • progressively reduced proliferation of granule cell precursors in cerebella starting at P5

♂ • increase in apoptosis of granule cell precursors in cerebella beginning at P7

♂ • cultured granule cell precurors show reduced proliferation that is largely restored with the addition of cholesterol to the culture medium

Genotype
MGI:3849179

cn35

• Allelic Composition: Nf1^tm1Fcr/Nf1^tm1Fcr; Trp53^tm1Elee/Trp53^tm1Elee; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129S/SvEv * 129S4/SvJae * FVB/N

mortality/aging

premature death ( J:149662 )

• all mice die prior to 200 days

neoplasm

increased glioma incidence ( J:149662 )

• 70% of gliomas exhibit necrosis, a feature of human glioblastoma multiforme

• tumors are relatively homogeneous histological and in lineage marker expression

decreased tumor latency ( J:149662 )

• all mice develop astrocytic gliomas with shorter latency than observed in Trp53^tm1Elee/Trp53^tm1Tyj Tg(GFAP-cre)25Mes or Trp53^tm1Elee/ Trp53^tm1Elee Tg(GFAP-cre)25Mes mice

nervous system

increased glioma incidence ( J:149662 )

• 70% of gliomas exhibit necrosis, a feature of human glioblastoma multiforme

• tumors are relatively homogeneous histological and in lineage marker expression

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
glioblastoma		DOID:3068		J:149662

Genotype
MGI:5560659

cn36

• Allelic Composition: Mef2a^tm1.1Limm/Mef2a^tm1.1Limm; Mef2d^tm3Eno/Mef2d^tm3Eno; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129S/SvEv * C57BL/6 * FVB/N

behavior/neurological

behavior/neurological phenotype ( J:207812 )

N • mice exhibit normal learning and memory in context- and cue-dependent fear conditioning tests, normal anxiety-related behaviors and pain sensitivity

impaired coordination ( J:207812 )

• on a rotarod

nervous system

nervous system phenotype ( J:207812 )

N • mice exhibit normal synapse number and basal synaptic transmission

enhanced paired-pulse facilitation ( J:207812 )

Genotype
MGI:4818691

cn37

• Allelic Composition: Mrtfb^tm2.1Eno/Mrtfb^tm2.1Eno; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129S/SvEv * C57BL/6 * FVB/N

normal phenotype

no abnormal phenotype detected ( J:161853 )

• mice are normal

Genotype
MGI:5560657

cn38

• Allelic Composition: Mef2a^tm1.1Limm/Mef2a^tm1.1Limm; Mef2c^tm2Eno/Mef2c^tm2Eno; Mef2d^tm3Eno/Mef2d^tm3Eno; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129S/SvEv * C57BL/6 * FVB/N

mortality/aging

premature death ( J:207812 )

• partial lethality by 5 weeks

nervous system

nervous system phenotype ( J:207812 )

N • mice exhibit normal numbers of spines per length of dendrite in pyramidal neurons

increased neuron apoptosis ( J:207812 )

• 5-fold

decreased brain size ( J:207812 )

abnormal long-term potentiation ( J:207812 )

• mice exhibit decreased input-output curve compared with wild-type mice

• however, long term potentiation is normal

enhanced paired-pulse facilitation ( J:207812 )

growth/size/body

decreased body weight ( J:207812 )

cellular

increased neuron apoptosis ( J:207812 )

• 5-fold

Genotype
MGI:5560660

cn39

• Allelic Composition: Mef2a^tm1.1Limm/Mef2a^tm1.1Limm; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129S/SvEv * C57BL/6 * FVB/N

behavior/neurological

behavior/neurological phenotype ( J:207812 )

N • mice exhibit normal behavior

Genotype
MGI:6275806

cn40

• Allelic Composition: Lgi1^tm2.1Jkc/Lgi1^tm2.1Jkc; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129/Sv * C57BL/6 * FVB/N

nervous system

nervous system phenotype ( J:269784 )

N • mice do not develop seizures, cortical dysplasia or hypercellularity in the outer cortical layers

Genotype
MGI:5286072

cn41

• Allelic Composition: Ptch1^tm1Bjw/Ptch1^tm1Bjw; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129T2/SvEms * FVB/N

mortality/aging

premature death ( J:139573 )

• by 4 weeks, mice become severely ill and must be sacrificed

nervous system

increased medulloblastoma incidence ( J:139573 )

abnormal cerebellum external granule cell layer morphology ( J:139573 )

• expanded at E16.5

• thick and disorganized, at birth

abnormal rhombic lip morphology ( J:139573 )

• rhombic lip expansion at E16.5

increased neuronal precursor cell number ( J:139573 )

• cerebellar cells exhibit increased neurospheres compared with wild-type mice

neoplasm

increased medulloblastoma incidence ( J:139573 )

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
medulloblastoma		DOID:0050902	OMIM:155255	J:139573

Genotype
MGI:3810317

cn42

• Allelic Composition: Gt(ROSA)26Sor^{tm1(Smo/EYFP)Amc}/Gt(ROSA)26Sor^{tm1(Smo/EYFP)Amc}; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129X1/SvJ * FVB/N

mortality/aging

premature death ( J:139574 )

• mice survive 57 days

neoplasm

increased medulloblastoma incidence ( J:139574 )

• mice develop diffuse medulloblastoma tumors and have a mean survival of 57 days

nervous system

increased medulloblastoma incidence ( J:139574 )

• mice develop diffuse medulloblastoma tumors and have a mean survival of 57 days

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
medulloblastoma		DOID:0050902	OMIM:155255	J:139574

Genotype
MGI:3641105

cn43

• Allelic Composition: Fgfr2^tm1Dor/Fgfr2^tm1Dor; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129X1/SvJ * FVB/N

nervous system

abnormal brain morphology ( J:110263 )

• mice show a subtle reduction in cortical size compared to control mice

abnormal astrocyte morphology ( J:110263 )

• numbers of astrocytes reaching the cortex is significantly reduced compared to controls at P7; greatest loss (60%) is in the upper cortical layers with 22% loss in the subcortical white matter

• there is an intermediate reduction (39%) in astrocte density was seen in the inferior cortical layers

Genotype
MGI:3641106

cn44

• Allelic Composition: Fgfr1^tm1Upir/Fgfr1^tm1Upir; Fgfr2^tm1Dor/Fgfr2^tm1Dor; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: 129X1/SvJ * FVB/N

nervous system

abnormal astrocyte morphology ( J:110263 )

• numbers of astrocytes reaching the cortex is significantly reduced compared to controls at P7

Genotype
MGI:5810136

cn45

• Allelic Composition: Bicd2^tm1Hgrd/Bicd2^tm1Hgrd; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: C57BL/6 * FVB/N

Disrupted laminar organization of the cortex in Bicd2^tm1Hgrd/Bicd2^tm1Hgrd Tg(GFAP-cre)25Mes/0 mice

mortality/aging

premature death ( J:210236 )

• mice with hydrocephalus die by 3-4 weeks of age

• mice without hydrocephalus survive to >8 weeks

nervous system

abnormal neuronal migration ( J:210236 )

• FoxP2, a marker normally present in deep corticofugal layer V and VI pyramidal neurons, is abnormally distributed in superficial lamina, consistent with impaired radial migration

• corticofugal axonal trajectories (labelled with anti-neurofilament M antibody) arise in superficial instead of deep cortical layers

abnormal cerebellar granule cell migration ( J:210236 )

• mice exhibit the same cerebellar granule cell migration defects observed in Bicd2^tm1.1Hgrd homozygotes

hydrocephaly ( J:210236 )

• some mice develop less severe hydrocephalus than that observed in Bicd2^tm1.1Hgrd mice

• others do not develop hydrocephalus

decreased corpus callosum size ( J:210236 )

• mice exhibit a thin corpus callosum

decreased brain external capsule size ( J:210236 )

• mice exhibit a thin capsula externa

abnormal hippocampus layer morphology ( J:210236 )

• mice without hydrocephalus exhibit disrupted laminar organization in the hippocampus

abnormal hippocampus pyramidal cell layer ( J:210236 )

• at P20, the hippocampus pyramidal cell layer appears disorganized in the CA1 region

abnormal stratification in cerebral cortex ( J:210236 )

• mice without hydrocephalus exhibit disrupted laminar organization in the cerebral cortex

• FoxP2, a marker normally present in deep corticofugal layer V and VI pyramidal neurons, is abnormally distributed in superficial lamina

• laminar organization of NeuN+ cells is absent

abnormal cerebellar layer morphology ( J:210236 )

• mice without hydrocephalus exhibit disrupted laminar organization in the cerebellar cortex

absent cerebellar granule layer ( J:210236 )

• mice do not develop an internal granule cell layer

cellular

abnormal neuronal migration ( J:210236 )

• FoxP2, a marker normally present in deep corticofugal layer V and VI pyramidal neurons, is abnormally distributed in superficial lamina, consistent with impaired radial migration

• corticofugal axonal trajectories (labelled with anti-neurofilament M antibody) arise in superficial instead of deep cortical layers

abnormal cerebellar granule cell migration ( J:210236 )

• mice exhibit the same cerebellar granule cell migration defects observed in Bicd2^tm1.1Hgrd homozygotes

Genotype
MGI:5609321

cn46

• Allelic Composition: Ephx2^tm1.1Arte/Ephx2^tm1.1Arte; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: C57BL/6 * FVB/N

cardiovascular system

abnormal retina vasculature morphology ( J:208344 )

• in the retina the upper capillary layer is less dense and characterized by fewer branching points in central areas and at the leading edge

• however, unlike in germ line deleted mice, no delay in radial extension of the vascular plexus is seen

vision/eye

abnormal retina vasculature morphology ( J:208344 )

• in the retina the upper capillary layer is less dense and characterized by fewer branching points in central areas and at the leading edge

• however, unlike in germ line deleted mice, no delay in radial extension of the vascular plexus is seen

Genotype
MGI:6719557

cn47

• Allelic Composition: Hip1^{tm5.1(HIP1)Tsr}/Hip1^{tm5.1(HIP1)Tsr}; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: C57BL/6 * FVB/N

skeleton

lordokyphosis ( J:267987 )

• unexpectedly, mice exhibit severe kypholordosis at 6 months of age, indicating that brain-specific expression of human HIP1 does not rescue the spinal phenotype

Genotype
MGI:5499900

cn48

• Allelic Composition: Drd2^tm1Smoc/Drd2^tm1Smoc; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: C57BL/6 * FVB/N

nervous system

nervous system phenotype ( J:194407 )

N • MPTP treated mice exhibit normal astrocyte proliferation in the substantia nigra

N • mice exhibit normal development of dopaminergic neurons in the ventral tagmental area

astrocytosis ( J:194407 )

• in the substantia nigra

abnormal astrocyte physiology ( J:194407 )

• mild increase in the production of pro-inflammatory mediator RNAs

Genotype
MGI:6256831

cn49

• Allelic Composition: Trpv4^{tm1c(KOMP)Wtsi}/Trpv4^{tm1c(KOMP)Wtsi}; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: C57BL/6J * C57BL/6N * FVB/N

homeostasis/metabolism

abnormal response to injury ( J:266383 )

• after region-specific injection of hyaluronic acid to induce acute retinal detachment (RD), Mueller cells exhibit 50% less cell death and fail to exhibit significant mechanical stimuli-evoked current or inward current and HC067047 did not have an inhibitory effect unlike in wild-type Mueller cells

• however, retina subjected to RD exhibit normal gliosis and increased proliferation

Genotype
MGI:5297540

cn50

• Allelic Composition: Aqp4^tm1.1Geno/Aqp4^tm1.1Geno; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: C57BL/6J * FVB/N

cardiovascular system

abnormal blood-brain barrier function ( J:177412 )

• after water injection, mice exhibit decreased water uptake compared with similarly treated wild-type mice

• basal brain water content is increased compared to in wild-type mice

• however, the blood-brain barrier is intake in a Evans dye transfer assay

nervous system

abnormal blood-brain barrier function ( J:177412 )

• after water injection, mice exhibit decreased water uptake compared with similarly treated wild-type mice

• basal brain water content is increased compared to in wild-type mice

• however, the blood-brain barrier is intake in a Evans dye transfer assay

Genotype
MGI:6377096

cn51

• Allelic Composition: Marveld1^tm1.1Liy/Marveld1^tm1.1Liy; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: C57BL/6J * FVB/N

nervous system

abnormal cerebellar granule cell migration ( J:279065 )

• granule cells accumulate on the molecular layer

abnormal cerebellar Purkinje cell layer ( J:279065 )

• irregular at 6 days of age

abnormal Purkinje cell dendrite morphology ( J:279065 )

• apical dendritic arbors are abnormal and unable to anchor in the basement membrane

abnormal CNS glial cell morphology ( J:279065 )

• absence of strong glial fibers in mice at 6 days of age

cellular

abnormal cerebellar granule cell migration ( J:279065 )

• granule cells accumulate on the molecular layer

Genotype
MGI:4939886

cn52

• Allelic Composition: Ren1^tm1.1Sig/Ren1^tm1.1Sig; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: C57BL/6J * FVB/N

mortality/aging

mortality/aging ( J:168753 )

N • mice exhibit normal survival unlike Ren1^tm1.2Sig homozygotes

renal/urinary system

renal/urinary system phenotype ( J:168753 )

N • mice exhibit normal renal morphology and function

cardiovascular system

cardiovascular system phenotype ( J:168753 )

N • mice exhibit normal blood pressure

homeostasis/metabolism

homeostasis/metabolism phenotype ( J:168753 )

N • mice exhibit normal metabolism

Genotype
MGI:4939155

cn53

• Allelic Composition: S1pr1^tm1Jch/S1pr1^tm1Jch; Tg(GFAP-cre)25Mes/?
• Genetic Background: involves: C57BL/6J * FVB/N

immune system

decreased susceptibility to experimental autoimmune encephalomyelitis ( J:168824 )

• Experimental Autoimmune Encephalitis induced by Myelin Oligodendrocyte Glycoprotein is attenuated but otherwise similar to contrtols

nervous system

astrocytosis ( J:168824 )

• does not develop in Experimental Autoimmune Encephalitis

abnormal axon morphology ( J:168824 )

• reduced axonal damage in Experimental Autoimmune Encephalitis

demyelination ( J:168824 )

• reduced in Experimental Autoimmune Encephalitis

Genotype
MGI:7612229

cn54

• Allelic Composition: Mldhr^tm1Zhgn/Mldhr^tm1Zhgn; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: FVB/N

neoplasm

increased glioblastoma incidence ( J:303725 )

• at 55-65 weeks of age

behavior/neurological

ataxia ( J:303725 )

• at 55-65 weeks of age

paralysis ( J:303725 )

• at 55-65 weeks of age

seizures ( J:303725 )

• at 55-65 weeks of age

nervous system

seizures ( J:303725 )

• at 55-65 weeks of age

increased glioblastoma incidence ( J:303725 )

• at 55-65 weeks of age

Genotype
MGI:3641103

cn55

• Allelic Composition: Fgfr1^tm1Upir/Fgfr1^tm1Upir; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: FVB/N

nervous system

abnormal neuronal migration ( J:110263 )

• in mutants, midline cells do not migrate from the ventricular zone to the subpial region of the dorsomedial pallium

abnormal brain commissure morphology ( J:110263 )

• average midline width of the dorsal commissures is markedly smaller compared to controls; anterior and posterior are significantly more affected than the middle

abnormal corpus callosum morphology ( J:110263 )

• in 83% of mice, there is a complete loss of callosal axons from the motor and somatosensory cortex at birth

abnormal hippocampal commissure morphology ( J:110263 )

• there is a loss of hippocampal commissural axons in neonatal mice

abnormal astrocyte morphology ( J:110263 )

• idusium griseum astroglia are absent in the anterior regions of mutants

• processes emerging from the glial wedge and ventricular zone remain attached at the ventricular zone and the pia and very few astrocytes are observed in between the glial wedge and the idusium griseum

• numbers of astrocytes reaching the cortex are significantly reduced compared to controls

cellular

abnormal neuronal migration ( J:110263 )

• in mutants, midline cells do not migrate from the ventricular zone to the subpial region of the dorsomedial pallium

Genotype
MGI:3612962

cn56

• Allelic Composition: Ntrk2^tm1Jom/Ntrk2^tm1Jom; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: FVB/N

nervous system

nervous system phenotype ( J:103935 )

N • no defects in barrel cortex formation

Genotype
MGI:6199477

cn57

• Allelic Composition: Lrp2^tm1Tew/Lrp2^tm1Tew; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: FVB/N

vision/eye

vision/eye phenotype ( J:238249 )

N • mice exhibit normal retinal differentiation and eye formation

Genotype
MGI:5513868

cn58

• Allelic Composition: Dio2^tm1Acb/Dio2^tm1Acb; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: FVB/N

skeleton

decreased bone mineral density ( J:197623 )

endocrine/exocrine glands

endocrine/exocrine gland phenotype ( J:197623 )

N • mice exhibit normal hypothalamic-pituitary-thyroid axis (HPT), HPT sensitivity and thyroid economy

Genotype
MGI:3770149

cn59

• Allelic Composition: Bdnf^tm1Limm/Bdnf^tm1Limm; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: FVB/N

behavior/neurological

abnormal depression-related behavior ( J:129526 )

• female, but not male, mice exhibit more immobility when placed in swim test than wild-type female mice

increased locomotor activity ( J:129526 )

• male, but not female, mice exhibit increased locomotor activity compared to same sex wild-type mice

Genotype
MGI:5467513

cn60

• Allelic Composition: Ric8a^tm1.1Zhua/Ric8a^tm1.1Zhua; Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: FVB/N

nervous system

abnormal cerebellar granule cell migration ( J:191222 )

• impaired migration from the external to internal granule layer

abnormal cerebellum morphology ( J:191222 )

• severely disorganized

abnormal cerebellum development ( J:191222 )

• no basement membrane separates neighboring lobules in the cerebellum and remains on the surface

• however, granule cell precursors are normal

abnormal cerebellar foliation ( J:191222 )

• no basement membrane separates neighboring lobules

• however, folial pattern is more or less normal

abnormal Bergmann glial cell morphology ( J:191222 )

• Bergmann glial scaffolds surrounding he primary and secondary fissures are severely disorganized

• the Bergmann glial scaffolds surrounding other fissures is severely reduced in fiber density compared to in wild-type mice

• severely reduced Bergmann glia-basement membrane interaction compared to in wild-type mice

• however, the Bergmann glial scaffolds surrounding other fissures is still associated with basement membrane

ectopic Purkinje cell ( J:191222 )

• scattered over a wide area in the cerebellum and frequently intermingled with ectopic granule cells

• most obvious in lobules surrounding the primary and secondary fissures

ectopic cerebellar granule cells ( J:191222 )

• throughout the molecular layer and external granule layer (EGL) surrounding the primary and secondary fissures

• in the EGL, but not the molecular layer, surrounding other lobules

absent cerebellum fissure ( J:191222 )

• primary and secondary fissures

abnormal cerebellum lobule morphology ( J:191222 )

• no basement membrane separates neighboring lobules

• however, folial pattern is more or less normal

enlarged cerebellum ( J:191222 )

• expanded volume from P0 to P4

behavior/neurological

ataxia ( J:191222 )

cellular

abnormal cerebellar granule cell migration ( J:191222 )

• impaired migration from the external to internal granule layer

abnormal basement membrane morphology ( J:191222 )

• no basement membrane separates neighboring lobules in the cerebellum and remains on the surface