All Phenotypes Tg(Dhh-cre)1Mejr MGI Mouse

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Phenotypes associated with this allele

Allele Symbol
Allele Name
Allele ID

Tg(Dhh-cre)1Mejr
transgene insertion 1, Dies Meijer
MGI:4359600

Summary

31 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Cdkn2a^tm1Rdp/Cdkn2a^tm1Rdp Meis1^tm2Ngc/Meis1^tm2Ngc Nf1^tm1Fcr/Nf1⁺ Tg(Dhh-cre)1Mejr/0	involves: 129 * C57BL/6 * C57BL/6J * FVB/N * SJL	MGI:8402603
cn2	Gata1^tm1Phi/Y Tg(Dhh-cre)1Mejr/0	involves: 129P2/OlaHsd * C57BL/6 * FVB	MGI:2677349
cn3	Erbb3^tm3Cbm/Erbb3^tm3Cbm Gt(ROSA)26Sor^{tm8(Map2k1*,-EGFP)Rsky}/? Tg(Dhh-cre)1Mejr/0	involves: 129P2/OlaHsd * C57BL/6 * FVB/N	MGI:5588163
cn4	Erbb3^tm3Cbm/Erbb3^tm3Cbm Gt(ROSA)26Sor^{tm7(Pik3ca*,-EGFP)Rsky}/? Tg(Dhh-cre)1Mejr/0	involves: 129P2/OlaHsd * C57BL/6 * FVB/N	MGI:5588164
cn5	Gt(ROSA)26Sor^{tm8(Map2k1*,-EGFP)Rsky}/? Ptpn11^tm1.1Wbm/Ptpn11^tm1.1Wbm Tg(Dhh-cre)1Mejr/0	involves: 129P2/OlaHsd * C57BL/6 * FVB/N	MGI:5588150
cn6	Gt(ROSA)26Sor^{tm7(Pik3ca*,-EGFP)Rsky}/? Ptpn11^tm1.1Wbm/Ptpn11^tm1.1Wbm Tg(Dhh-cre)1Mejr/0	involves: 129P2/OlaHsd * C57BL/6 * FVB/N	MGI:5588151
cn7	Sox10^{tm7.1(Sox10)Weg}/Sox10^{tm7.1(Sox10)Weg} Tg(Dhh-cre)1Mejr/0	involves: 129P2/OlaHsd * C57BL/6 * FVB/N * SJL	MGI:4820434
cn8	Erbb3^tm3Cbm/Erbb3^tm3Cbm Tg(Dhh-cre)1Mejr/0	involves: 129P2/OlaHsd * FVB/N	MGI:5588162
cn9	Ptpn11^tm1.1Wbm/Ptpn11^tm1.1Wbm Tg(Dhh-cre)1Mejr/0	involves: 129P2/OlaHsd * FVB/N	MGI:5588149
cn10	Pou3f1^tm1Mejr/Pou3f1^tm2.1Mejr Pou3f2^tm1Mejr/Pou3f2^tm1Mejr Tg(Dhh-cre)1Mejr/0	involves: 129P2/OlaHsd * FVB/N	MGI:4359761
cn11	Pou3f2^tm1Mejr/Pou3f2^tm1Mejr Tg(Dhh-cre)1Mejr/0	involves: 129P2/OlaHsd * FVB/N	MGI:4359759
cn12	Dmrtc2^tm1.1Zark/Dmrtc2^tm1.1Zark Tg(Dhh-cre)1Mejr/0	involves: 129S1/Sv * C57BL/6	MGI:3711737
cn13	Pten^tm2.1Ppp/Pten^tm2.1Ppp Tg(Cnp-EGFR)10Nrat/0 Tg(Dhh-cre)1Mejr/0	involves: 129S1/Sv * C57BL/6 * FVB/N * SJL	MGI:5485414
cn14	Pten^tm2.1Ppp/Pten^tm2.1Ppp Tg(Dhh-cre)1Mejr/0	involves: 129S1/Sv * C57BL/6 * FVB/N * SJL	MGI:5485416
cn15	Pten^tm2.1Ppp/Pten⁺ Tg(Cnp-EGFR)10Nrat/0 Tg(Dhh-cre)1Mejr/0	involves: 129S1/Sv * C57BL/6 * FVB/N * SJL	MGI:5485415
cn16	Septin9^tm2.1Emfu/Septin9^tm2.1Emfu Tg(Dhh-cre)1Mejr/0	involves: 129S1/Sv * FVB/N	MGI:7523323
cn17	Cmtm6^{tm1c(EUCOMM)Wtsi}/Cmtm6^{tm1c(EUCOMM)Wtsi} Mag^tm1Mtg/Mag^tm1Mtg Tg(Dhh-cre)1Mejr/0	involves: 129S2/SvPas * 129S4/SvJaeSor * C57BL/6N * FVB/N	MGI:6511254
cn18	Nf1^tm1.1Kest/Nf1^{tm1c(KOMP)Wtsi} Tg(Dhh-cre)1Mejr/0	involves: 129S4/SvJaeSor * C57BL/6 * C57BL/6N * C57BL/6NTac * FVB/N	MGI:6275135
cn19	Nf1^{tm1c(KOMP)Wtsi}/Nf1^{tm1c(KOMP)Wtsi} Tg(Dhh-cre)1Mejr/0	involves: 129S4/SvJaeSor * C57BL/6 * C57BL/6N * FVB/N	MGI:6275138
cn20	Cmtm6^{tm1c(EUCOMM)Wtsi}/Cmtm6^{tm1c(EUCOMM)Wtsi} Tg(Dhh-cre)1Mejr/0	involves: 129S4/SvJaeSor * C57BL/6N * FVB/N	MGI:6511251
cn21	Septin2^{tm1c(EUCOMM)Wtsi}/Septin2^{tm1c(EUCOMM)Wtsi} Tg(Dhh-cre)1Mejr/0	involves: 129S4/SvJaeSor * C57BL/6N * FVB/N	MGI:7523329
cn22	Lgi4^tm1.1Jrb/Lgi4^tm1.1Jrb Tg(Dhh-cre)1Mejr/0	involves: 129S4/SvJaeSor * FVB/N	MGI:4441321
cn23	Adam22^tm1.1Mejr/Adam22^tm1.1Mejr Tg(Dhh-cre)1Mejr/0	involves: 129S4/SvJaeSor * FVB/N	MGI:4441315
cn24	Slc12a6^tm2.1Dlp/Slc12a6^tm2.1Dlp Tg(Dhh-cre)1Mejr/0	involves: 129S6/SvEvTac * C57BL/6J * FVB/N	MGI:5754619
cn25	Cadm4^tm1.1Pele/Cadm4^tm1.1Pele Tg(Dhh-cre)1Mejr/0	involves: 129S/SvEv * 129S4/SvJaeSor * FVB/N	MGI:5528812
cn26	Dmrt1^tm1Zark/Dmrt1^tm1.1Zark Tg(Dhh-cre)1Mejr/0	involves: 129/Sv * C57BL/6	MGI:3851610
cn27	Gt(ROSA)26Sor^{tm8(Map2k1*,-EGFP)Rsky}/? Tg(Dhh-cre)1Mejr/0	involves: C57BL/6 * FVB/N	MGI:5588152
cn28	Cemip^tm1.1Tac/Cemip^tm1.1Tac Tg(Dhh-cre)1Mejr/0	involves: C57BL/6NTac * FVB/N	MGI:6117715
cn29	Fgd4^tm1Ics/Fgd4^tm1Ics Tg(Dhh-cre)1Mejr/0	involves: FVB/N	MGI:5460880
cn30	Adam23^tm1.1Mejr/Adam23^tm1.1Mejr Tg(Dhh-cre)1Mejr/0	involves: FVB/N	MGI:7444421
cx31	Cdkn2a^tm1Rdp/Cdkn2a^tm1Rdp Nf1^tm1Fcr/Nf1⁺ Tg(Dhh-cre)1Mejr/0	B6.Cg-Cdkn2a^tm1Rdp Nf1^tm1Fcr Tg(Dhh-cre)1Mejr	MGI:8402593

Allelic Composition	Cdkn2a^tm1Rdp/Cdkn2a^tm1Rdp Meis1^tm2Ngc/Meis1^tm2Ngc Nf1^tm1Fcr/Nf1⁺ Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129 * C57BL/6 * C57BL/6J * FVB/N * SJL

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cdkn2a^tm1Rdp mutation (3 available); any Cdkn2a mutation (60 available)
Meis1^tm2Ngc mutation (1 available); any Meis1 mutation (46 available)
Nf1^tm1Fcr mutation (1 available); any Nf1 mutation (160 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

mortality/aging

premature death ( J:240370 )

• mice succumb to hematopoietic malignancies, with enlarged liver and/or spleen

neoplasm

decreased sarcoma incidence ( J:240370 )

• fewer malignant peripheral nerve sheath tumors are seen compared to mice that do not have Meis1 deleted in nerve glial cells; these tumors stain positive for Meis1, suggesting that they develop in nerve glial cells that escapade inactivation of Meis1

increased hemolymphoid system tumor incidence ( J:240370 )

• about 66% of mice develop hematopoietic neoplasms

• mice that fail to develop malignant peripheral nerve sheath tumors develop hematopoietic disease as indicated by an increase in liver/spleen size

Allelic Composition	Gata1^tm1Phi/Y Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129P2/OlaHsd * C57BL/6 * FVB

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gata1^tm1Phi mutation (0 available); any Gata1 mutation (47 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

normal phenotype

no abnormal phenotype detected ( J:85598 )

• these mice were fertile and exhibited normal testicular development and spermatogenesis

Allelic Composition	Erbb3^tm3Cbm/Erbb3^tm3Cbm Gt(ROSA)26Sor^{tm8(Map2k1*,-EGFP)Rsky}/? Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129P2/OlaHsd * C57BL/6 * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Erbb3^tm3Cbm mutation (0 available); any Erbb3 mutation (46 available)
Gt(ROSA)26Sor^{tm8(Map2k1*,-EGFP)Rsky} mutation (1 available); any Gt(ROSA)26Sor mutation (1209 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

nervous system

decreased myelin sheath thickness ( J:207470 )

• at P15, the myelin of peripheral axons is thinner but axons are hypermyelinated at P90

abnormal myelination ( J:207470 )

• at P15 and P90, 36.7% and 17.8% of axons with diameters >1 um are nonmyelinated, respectively compared to 3.5% and 0.8% in controls

hypermyelination ( J:207470 )

• axons are hypermyelinated at P90

• however, mice show rescue of Schwann cell development and Schwan cells are able to myelinate

Allelic Composition	Erbb3^tm3Cbm/Erbb3^tm3Cbm Gt(ROSA)26Sor^{tm7(Pik3ca*,-EGFP)Rsky}/? Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129P2/OlaHsd * C57BL/6 * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Erbb3^tm3Cbm mutation (0 available); any Erbb3 mutation (46 available)
Gt(ROSA)26Sor^{tm7(Pik3ca*,-EGFP)Rsky} mutation (1 available); any Gt(ROSA)26Sor mutation (1209 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

nervous system

abnormal myelination ( J:207470 )

• myelination is not seen

Allelic Composition	Gt(ROSA)26Sor^{tm8(Map2k1*,-EGFP)Rsky}/? Ptpn11^tm1.1Wbm/Ptpn11^tm1.1Wbm Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129P2/OlaHsd * C57BL/6 * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sor^{tm8(Map2k1*,-EGFP)Rsky} mutation (1 available); any Gt(ROSA)26Sor mutation (1209 available)
Ptpn11^tm1.1Wbm mutation (1 available); any Ptpn11 mutation (45 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

nervous system

hypermyelination ( J:207470 )

• axons of peripheral nerves are hypermyelinated at P90

• however, mice show normal numbers of Schwann cells

Allelic Composition	Gt(ROSA)26Sor^{tm7(Pik3ca*,-EGFP)Rsky}/? Ptpn11^tm1.1Wbm/Ptpn11^tm1.1Wbm Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129P2/OlaHsd * C57BL/6 * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sor^{tm7(Pik3ca*,-EGFP)Rsky} mutation (1 available); any Gt(ROSA)26Sor mutation (1209 available)
Ptpn11^tm1.1Wbm mutation (1 available); any Ptpn11 mutation (45 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

nervous system

abnormal myelination ( J:207470 )

• mice show myelination defects similar to single conditional Ptpn11^tm1.1Wbm homozygotes

Allelic Composition	Sox10^{tm7.1(Sox10)Weg}/Sox10^{tm7.1(Sox10)Weg} Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129P2/OlaHsd * C57BL/6 * FVB/N * SJL

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Sox10^{tm7.1(Sox10)Weg} mutation (0 available); any Sox10 mutation (36 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

mortality/aging

premature death ( J:162692 )

• most mice die by 5 weeks of age

• all mice die by 7 weeks of age

nervous system

abnormal Schwann cell morphology ( J:162692 )

• as determined by marker expression, Schwann cells do not progress beyond the immature stage unlike wild-type cells

• between P8 and P32, the number of Schwann cells in the sciatic nerve is increased slightly compared to in wild-type mice

• Schwann cells contain high amounts of rough endoplasmic reticulum compared to in wild-type mice

increased neuron apoptosis ( J:162692 )

• increased in the sciatic nerve

abnormal neuron proliferation ( J:162692 )

• increased in the sciatic nerve

abnormal sciatic nerve morphology ( J:162692 )

• sciatic nerves are translucent, less well defined, lacks myelination, and lacks the characteristic banded pattern compared with sciatic nerves from wild-type mice

• the number of cells in the sciatic nerve is increased compared to in wild-type mice

• between P8 and P32, the number of Schwann cells in the sciatic nerve is increased slightly compared to in wild-type mice

• from P16 to P32, sciatic nerves contain increased endothelial cells, pericytes, macrophages, T lymphocytes, perineurial cells, endoneurial fibroblast, and adipocytes compared in wild-type mice

• sciatic nerves contain a dense layer of collagen and elastic fibers unlike in wild-type mice

• at P16 and P32, Remak bundles within the sciatic nerve are absent unlike in wild-type mice

• the perineural sheath is thinner than in wild-type mice

abnormal myelination ( J:162692 )

• in the sciatic nerve

abnormal action potential ( J:162692 )

• sciatic nerves compound action potentials lack the fast component unlike in wild-type mice

decreased nerve conduction velocity ( J:162692 )

• in the sciatic nerve

behavior/neurological

tremors ( J:162692 )

• shiverer-type

impaired coordination ( J:162692 )

abnormal limb posture ( J:162692 )

• mice develop a straddled position of the hindlimbs unlike wild-type mice

abnormal locomotor behavior ( J:162692 )

• mice exhibit progressive deterioration of locomotion unlike wild-type mice

abnormal gait ( J:162692 )

• staggering gait

growth/size/body

decreased body weight ( J:162692 )

• beginning at P8

• 52% lighter at P24

• 68% lighter at P32

postnatal growth retardation ( J:162692 )

• mice fail to thrive shortly after birth

cellular

abnormal Schwann cell morphology ( J:162692 )

• as determined by marker expression, Schwann cells do not progress beyond the immature stage unlike wild-type cells

• between P8 and P32, the number of Schwann cells in the sciatic nerve is increased slightly compared to in wild-type mice

• Schwann cells contain high amounts of rough endoplasmic reticulum compared to in wild-type mice

increased neuron apoptosis ( J:162692 )

• increased in the sciatic nerve

abnormal neuron proliferation ( J:162692 )

• increased in the sciatic nerve

Allelic Composition	Erbb3^tm3Cbm/Erbb3^tm3Cbm Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129P2/OlaHsd * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Erbb3^tm3Cbm mutation (0 available); any Erbb3 mutation (46 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

nervous system

absent Schwann cells ( J:207470 )

• almost complete absence of Schwann cells

cellular

absent Schwann cells ( J:207470 )

• almost complete absence of Schwann cells

Allelic Composition	Ptpn11^tm1.1Wbm/Ptpn11^tm1.1Wbm Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129P2/OlaHsd * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Ptpn11^tm1.1Wbm mutation (1 available); any Ptpn11 mutation (45 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

nervous system

decreased Schwann cell number ( J:207470 )

abnormal Schwann cell physiology ( J:207470 )

• disruption of entry into the myelination program of remaining Schwann cells

cellular

decreased Schwann cell number ( J:207470 )

abnormal Schwann cell physiology ( J:207470 )

• disruption of entry into the myelination program of remaining Schwann cells

Allelic Composition	Pou3f1^tm1Mejr/Pou3f1^tm2.1Mejr Pou3f2^tm1Mejr/Pou3f2^tm1Mejr Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129P2/OlaHsd * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pou3f1^tm1Mejr mutation (0 available); any Pou3f1 mutation (14 available)
Pou3f1^tm2.1Mejr mutation (0 available); any Pou3f1 mutation (14 available)
Pou3f2^tm1Mejr mutation (0 available); any Pou3f2 mutation (23 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

nervous system

abnormal axon morphology ( J:83739 )

• at P56 and P120 (about 55% and 25%), respectively, of nerves are abnormal with many promyelin configurations

abnormal myelination ( J:83739 )

• at P16 nerves contain many promyelin configurations in contrast to wild type and heterozygotes in which >90% of large-caliber axons are myelinated

• at P56 and P120, abnormal axons are thinly myelinated resembling wild-type nerves during the first postnatal week

Allelic Composition	Pou3f2^tm1Mejr/Pou3f2^tm1Mejr Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129P2/OlaHsd * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pou3f2^tm1Mejr mutation (0 available); any Pou3f2 mutation (23 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

nervous system

nervous system phenotype ( J:83739 )

N	• animals are normal and healthy; sciatic nerves of P8 mice show no obvious morphological abnormalities

Allelic Composition	Dmrtc2^tm1.1Zark/Dmrtc2^tm1.1Zark Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129S1/Sv * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmrtc2^tm1.1Zark mutation (1 available); any Dmrtc2 mutation (13 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

endocrine/exocrine glands

small testis ( J:121537 )

• testis size is slightly decreased in P14 mutants

reproductive system

reproductive system phenotype ( J:121537 )

N	• spermatogenesis is normal; Sertoli cell location is normal

small testis ( J:121537 )

• testis size is slightly decreased in P14 mutants

Allelic Composition	Pten^tm2.1Ppp/Pten^tm2.1Ppp Tg(Cnp-EGFR)10Nrat/0 Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129S1/Sv * C57BL/6 * FVB/N * SJL

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pten^tm2.1Ppp mutation (0 available); any Pten mutation (83 available)
Tg(Cnp-EGFR)10Nrat mutation (0 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

mortality/aging

premature death ( J:195067 )

• rapid postnatal death, with a medial survival age of 26 days

nervous system

abnormal nervous system morphology ( J:195067 )

• mutants exhibit enlarged peripheral nerves: brachial plexi, sacral plexi, trigeminal and sciatic nerves

• 100% exhibit enlarged branchial plexi

• 50% exhibit enlarged lumbar sacral plexi

• mast cells are seen in enlarged peripheral nerves

increased neurofibrosarcoma incidence ( J:195067 )

• enlarged peripheral nerves are graded as high-grade grade peripheral nerve sheath tumors (PNSTs) resembling human sporatic malignant grade peripheral nerve sheath tumors

abnormal trigeminal nerve morphology ( J:195067 )

• 92% exhibit enlarged trigeminal nerves

enlarged dorsal root ganglion ( J:195067 )

• mutants exhibit multiple enlarged dorsal root ganglia

abnormal sciatic nerve morphology ( J:195067 )

• 50% exhibit enlarged sciatic nerves

neoplasm

increased neurofibrosarcoma incidence ( J:195067 )

• enlarged peripheral nerves are graded as high-grade grade peripheral nerve sheath tumors (PNSTs) resembling human sporatic malignant grade peripheral nerve sheath tumors

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
malignant peripheral nerve sheath tumor		DOID:5940		J:195067

Allelic Composition	Pten^tm2.1Ppp/Pten^tm2.1Ppp Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129S1/Sv * C57BL/6 * FVB/N * SJL

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Pten^tm2.1Ppp mutation (0 available); any Pten mutation (83 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

mortality/aging

premature death ( J:195067 )

• median survival age is 247 days compared to conditional Pten heterozygotes that have a median survival age of 415 days

nervous system

abnormal nervous system morphology ( J:195067 )

• mutants exhibit a similar peripheral nervous system phenotype as triple homozygous Pten^tm2.1Ppp Tg(Dhh-cre)1Mejr/0 Tg(Cnp-EGFR)10Nrat/0 mutants but with delayed latency and reduced tumor multiplicity

• 100% exhibit enlarged branchial plexi

• 10% exhibit enlarged sacral plexi

increased neurofibrosarcoma incidence ( J:195067 )

• enlarged peripheral nerves are low-grade peripheral nerve sheath tumors (PNSTs)

abnormal trigeminal nerve morphology ( J:195067 )

• 90% exhibit enlarged trigeminal nerves

abnormal sciatic nerve morphology ( J:195067 )

• 80% exhibit enlarged sciatic nerves

neoplasm

increased neurofibrosarcoma incidence ( J:195067 )

• enlarged peripheral nerves are low-grade peripheral nerve sheath tumors (PNSTs)

Allelic Composition	Pten^tm2.1Ppp/Pten⁺ Tg(Cnp-EGFR)10Nrat/0 Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129S1/Sv * C57BL/6 * FVB/N * SJL

Find Mice

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

nervous system

abnormal nervous system morphology ( J:195067 )

• mice display a similar peripheral nervous system phenotype as homozygous Pten^tm2.1Ppp Tg(Dhh-cre)1Mejr/0 mice, except with a latency of 415 days

• 100% exhibit enlarged branchial plexi

• 20% exhibit enlarged sacral plexi

increased neurofibrosarcoma incidence ( J:195067 )

• enlarged peripheral nerves are graded as hyperplasia to low-grade grade peripheral nerve sheath tumors (PNSTs)

abnormal trigeminal nerve morphology ( J:195067 )

• 100% exhibit enlarged trigeminal nerves

abnormal sciatic nerve morphology ( J:195067 )

• 80% exhibit enlarged sciatic nerves

neoplasm

increased neurofibrosarcoma incidence ( J:195067 )

• enlarged peripheral nerves are graded as hyperplasia to low-grade grade peripheral nerve sheath tumors (PNSTs)

Allelic Composition	Septin9^tm2.1Emfu/Septin9^tm2.1Emfu Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129S1/Sv * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Septin9^tm2.1Emfu mutation (0 available); any Septin9 mutation (202 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

nervous system

nervous system phenotype ( J:339068 )

• although the abundance of SEPTIN9 is markedly reduced in sciatic nerve lysates as expected, the abundance and localization of other septin subunits expressed in PNS myelin (SEPTIN2, SEPTIN7, SEPTIN8 and SEPTIN11) are similar to those in sciatic nerve lysates of control mice

• P14 sciatic nerves show no significant change in the number of normal-appearing myelinated axons; no axonal degeneration is noted and the number of myelin outfoldings is similar to that in control mice, indicating normal biogenesis of PNS myelin

• P75 sciatic nerves show similar abundance of myelin proteins (MAG, CNP, P0/MPZ, PMP22) relative to control mice

• mice exhibit normal motor nerve conduction velocity (mNCV) in sciatic nerves and normal sensory nerve conduction velocity (sNCV) in the tail nerve at 6 months of age

behavior/neurological

behavior/neurological phenotype ( J:339068 )

N	• mice show no alterations in the latency of retracting a hindpaw upon a mechanical stimulus (plantar test) at P45 or in the latency of falling from an accelerating rotating rod at 2.5 months of age

Allelic Composition	Cmtm6^{tm1c(EUCOMM)Wtsi}/Cmtm6^{tm1c(EUCOMM)Wtsi} Mag^tm1Mtg/Mag^tm1Mtg Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129S2/SvPas * 129S4/SvJaeSor * C57BL/6N * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cmtm6^{tm1c(EUCOMM)Wtsi} mutation (0 available); any Cmtm6 mutation (9 available)
Mag^tm1Mtg mutation (0 available); any Mag mutation (25 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

nervous system

abnormal axon morphology ( J:296495 )

• increased diameter of sciatic nerve axons

Allelic Composition	Nf1^tm1.1Kest/Nf1^{tm1c(KOMP)Wtsi} Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129S4/SvJaeSor * C57BL/6 * C57BL/6N * C57BL/6NTac * FVB/N
Cell Lines	EPD0033_1_F12

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nf1^tm1.1Kest mutation (0 available); any Nf1 mutation (160 available)
Nf1^{tm1c(KOMP)Wtsi} mutation (0 available); any Nf1 mutation (160 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

behavior/neurological

lethargy ( J:234172 )

hindlimb paralysis ( J:234172 )

• paralysis in one or both hind limbs begins at approximately 4 months of age, with 55.6% of mice showing paralysis by 5 months of age

growth/size/body

weight loss ( J:234172 )

homeostasis/metabolism

dehydration ( J:234172 )

immune system

dermatitis ( J:234172 )

integument

dermatitis ( J:234172 )

neoplasm

increased neurofibroma incidence ( J:234172 )

• 81.5% of mice exhibit neurofibroma tumors; tumors arise at the dorsal root ganglia, with invasion of the neural foramen and lead to compression of the spinal cord

• most tumors are detected in cervical and thoracic spine, with a lower percentage in the lumbar spine

• neurofibromas are characterized by disorganized Remak bundles and enriched deposition of collagen

nervous system

increased neurofibroma incidence ( J:234172 )

• 81.5% of mice exhibit neurofibroma tumors; tumors arise at the dorsal root ganglia, with invasion of the neural foramen and lead to compression of the spinal cord

• most tumors are detected in cervical and thoracic spine, with a lower percentage in the lumbar spine

• neurofibromas are characterized by disorganized Remak bundles and enriched deposition of collagen

abnormal somatic nervous system morphology ( J:234172 )

• enlarged peripheral nerves

abnormal spinal nerve morphology ( J:234172 )

• enlarged spinal nerve roots

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
neurofibromatosis 1		DOID:0111253	OMIM:162200	J:234172

Allelic Composition	Nf1^{tm1c(KOMP)Wtsi}/Nf1^{tm1c(KOMP)Wtsi} Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129S4/SvJaeSor * C57BL/6 * C57BL/6N * FVB/N
Cell Lines	EPD0033_1_F12

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nf1^{tm1c(KOMP)Wtsi} mutation (0 available); any Nf1 mutation (160 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

behavior/neurological

lethargy ( J:234172 )

hindlimb paralysis ( J:234172 )

• paralysis in one or both hind limbs begins at approximately 4 months of age, with 54.1% of mice showing paralysis by 5 months of age

growth/size/body

weight loss ( J:234172 )

homeostasis/metabolism

dehydration ( J:234172 )

immune system

dermatitis ( J:234172 )

integument

dermatitis ( J:234172 )

neoplasm

increased neurofibroma incidence ( J:234172 )

• 79.2% of mice exhibit neurofibroma tumors; tumors arise at the dorsal root ganglia, with invasion of the neural foramen and lead to compression of the spinal cord

• most tumors are detected in cervical and thoracic spine, with a lower percentage in the lumbar spine

• neurofibromas are characterized by disorganized Remak bundles and enriched deposition of collagen

nervous system

increased neurofibroma incidence ( J:234172 )

• 79.2% of mice exhibit neurofibroma tumors; tumors arise at the dorsal root ganglia, with invasion of the neural foramen and lead to compression of the spinal cord

• most tumors are detected in cervical and thoracic spine, with a lower percentage in the lumbar spine

• neurofibromas are characterized by disorganized Remak bundles and enriched deposition of collagen

abnormal somatic nervous system morphology ( J:234172 )

• enlarged peripheral nerves

abnormal spinal nerve morphology ( J:234172 )

• enlarged spinal nerve roots and lesions in sciatic nerves showing abnormal nonmyelinating axons

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
neurofibromatosis 1		DOID:0111253	OMIM:162200	J:234172

Allelic Composition	Cmtm6^{tm1c(EUCOMM)Wtsi}/Cmtm6^{tm1c(EUCOMM)Wtsi} Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129S4/SvJaeSor * C57BL/6N * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cmtm6^{tm1c(EUCOMM)Wtsi} mutation (0 available); any Cmtm6 mutation (9 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

nervous system

nervous system phenotype ( J:296495 )

N	• normal internode length and nodal and paranodal dimensions • normal neurofilament density and phosphorylation

abnormal axon morphology ( J:296495 )

• increased diameter of phrenic and ventral tail nerve axons

• increased diameter of non-myelinated sciatic nerve axons in Remak bundles at ages P9 and 2 months

• normal myelin sheath thickness to axon diameter ratio for phrenic and ventral tail nerve axons

• normal number of axons per Remak bundle, with no axon diameters larger than 1 micrometer

• normal frequency of developmentally sorted promyelinated axons

abnormal action potential ( J:296495 )

• increased sensory nerve action potential (SNAP)

increased nerve conduction velocity ( J:296495 )

• increased sensory nerve conduction velocity (SNCV)

behavior/neurological

abnormal gait ( J:296495 )

• increased slipping when walking over a regular grid

decreased thermal nociceptive threshold ( J:296495 )

• accelerated reaction in hot plate test

respiratory system

abnormal pulmonary respiratory rate ( J:296495 )

• absence of spontaneous apnea

Allelic Composition	Septin2^{tm1c(EUCOMM)Wtsi}/Septin2^{tm1c(EUCOMM)Wtsi} Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129S4/SvJaeSor * C57BL/6N * FVB/N
Cell Lines	EPD0175_4_D03

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Septin2^{tm1c(EUCOMM)Wtsi} mutation (0 available); any Septin2 mutation (58 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

nervous system

nervous system phenotype ( J:339068 )

• P75 sciatic nerves show similar abundance of myelin proteins (MAG, CNP, P0/MPZ, PMP22) relative to control mice

• motor nerve conduction velocity (mNCV) in sciatic nerves and sensory nerve conduction velocity (sNCV) in the tail nerve are slightly but not significantly decreased at 1 year of age

abnormal sciatic nerve morphology ( J:339068 )

• while the abundance of SEPTIN2 is markedly reduced in sciatic nerve lysates as expected, the abundance of other septin subunits expressed in PNS myelin (SEPTIN7, SEPTIN8, SEPTIN9, and SEPTIN11) is also substantially lower than that in the sciatic nerve lysates of control mice

behavior/neurological

behavior/neurological phenotype ( J:339068 )

• mice show no alterations in the latency of retracting a hindpaw upon a mechanical stimulus (plantar test) at 32 weeks of age or in the latency of falling from an accelerating rotating rod at 6 months of age

Allelic Composition	Lgi4^tm1.1Jrb/Lgi4^tm1.1Jrb Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129S4/SvJaeSor * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lgi4^tm1.1Jrb mutation (0 available); any Lgi4 mutation (32 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

nervous system

demyelination ( J:158973 )

• in the sciatic nerve at P12 similar to Lgi4^clp homozygotes

Allelic Composition	Adam22^tm1.1Mejr/Adam22^tm1.1Mejr Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129S4/SvJaeSor * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Adam22^tm1.1Mejr mutation (0 available); any Adam22 mutation (71 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

nervous system

nervous system phenotype ( J:158973 )

N	• motor neuron myelination is normal

Allelic Composition	Slc12a6^tm2.1Dlp/Slc12a6^tm2.1Dlp Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129S6/SvEvTac * C57BL/6J * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Slc12a6^tm2.1Dlp mutation (0 available); any Slc12a6 mutation (117 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

behavior/neurological

behavior/neurological phenotype ( J:217087 )

N	• mice display no locomotor deficit in the accelerated rotarod and open field tests relative to control mice

Allelic Composition	Cadm4^tm1.1Pele/Cadm4^tm1.1Pele Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129S/SvEv * 129S4/SvJaeSor * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cadm4^tm1.1Pele mutation (0 available); any Cadm4 mutation (13 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

nervous system

abnormal myelin sheath morphology ( J:199632 )

• myelin abnormalities including tomacula, redundant myelin, and outfolds

Allelic Composition	Dmrt1^tm1Zark/Dmrt1^tm1.1Zark Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: 129/Sv * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Dmrt1^tm1.1Zark mutation (0 available); any Dmrt1 mutation (47 available)
Dmrt1^tm1Zark mutation (0 available); any Dmrt1 mutation (47 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

endocrine/exocrine glands

abnormal testis development ( J:122954 )

• at P9 Sertoli cells are randomly distributed unlike in controls

• at P14 tubules lack luminal space and are instead filled with a poorly organized mix of Sertoli cells and differentiating germ cells

• at P28 tubules contain no apparent germ cells and are filled with randomized, unpolarized Sertoli cells with immature morphology

abnormal Sertoli cell development ( J:122954 )

• Sertoli cells fail to migrate properly and retain an immature morphology at P28

reproductive system

abnormal spermatogonia morphology ( J:122954 )

• by P9 the distribution of germs cells is highly abnormal with more cells in the center and fewer cells at the periphery of the tubules

• meiosis initiates but germ cells are absent by P28

abnormal testis development ( J:122954 )

• at P9 Sertoli cells are randomly distributed unlike in controls

• at P14 tubules lack luminal space and are instead filled with a poorly organized mix of Sertoli cells and differentiating germ cells

• at P28 tubules contain no apparent germ cells and are filled with randomized, unpolarized Sertoli cells with immature morphology

abnormal Sertoli cell development ( J:122954 )

• Sertoli cells fail to migrate properly and retain an immature morphology at P28

cellular

abnormal spermatogonia morphology ( J:122954 )

• by P9 the distribution of germs cells is highly abnormal with more cells in the center and fewer cells at the periphery of the tubules

• meiosis initiates but germ cells are absent by P28

Allelic Composition	Gt(ROSA)26Sor^{tm8(Map2k1*,-EGFP)Rsky}/? Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: C57BL/6 * FVB/N

Find Mice

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

nervous system

nervous system phenotype ( J:207470 )

N	• rarely observe aberrantly myelinated small axons in mutants and the ratio of nonmyelinated to myelinated axons is unchanged

Allelic Composition	Cemip^tm1.1Tac/Cemip^tm1.1Tac Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: C57BL/6NTac * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cemip^tm1.1Tac mutation (0 available); any Cemip mutation (70 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

nervous system

decreased susceptibility to nerve crush injury ( J:244311 )

• following nerve axotomy or sciatic nerve crush, mice exhibit reduced myelin degradation and increases remyelination compared with wild-type mice

• however, sciatic function index and Von Frey threshold are normal after nerve crush

abnormal myelination ( J:244311 )

• transient acceleration of postnatal myelination

• however, adult myelination is normal

homeostasis/metabolism

decreased susceptibility to nerve crush injury ( J:244311 )

• following nerve axotomy or sciatic nerve crush, mice exhibit reduced myelin degradation and increases remyelination compared with wild-type mice

• however, sciatic function index and Von Frey threshold are normal after nerve crush

Allelic Composition	Fgd4^tm1Ics/Fgd4^tm1Ics Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fgd4^tm1Ics mutation (0 available); any Fgd4 mutation (79 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

nervous system

abnormal myelination ( J:190437 )

• aberrant myelin formation and myelin in peripheral nerves

Allelic Composition	Adam23^tm1.1Mejr/Adam23^tm1.1Mejr Tg(Dhh-cre)1Mejr/0
Genetic Background	involves: FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Adam23^tm1.1Mejr mutation (0 available); any Adam23 mutation (62 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

nervous system

nervous system phenotype ( J:333962 )

N	• mice exhibit normal Kv1 channel complex protein at the juxtaparanodal membrane in sciatic nerves

Allelic Composition	Cdkn2a^tm1Rdp/Cdkn2a^tm1Rdp Nf1^tm1Fcr/Nf1⁺ Tg(Dhh-cre)1Mejr/0
Genetic Background	B6.Cg-Cdkn2a^tm1Rdp Nf1^tm1Fcr Tg(Dhh-cre)1Mejr

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cdkn2a^tm1Rdp mutation (3 available); any Cdkn2a mutation (60 available)
Nf1^tm1Fcr mutation (1 available); any Nf1 mutation (160 available)
Tg(Dhh-cre)1Mejr mutation (1 available)

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

mortality/aging

premature death ( J:240370 )

• mice die by 10 months of age

• mice succumb to hematopoietic malignancies

neoplasm

increased neurofibrosarcoma incidence ( J:240370 )

• malignant peripheral nerve sheath tumors are evident typically at 4-6 months of age on the shoulders, ribs, or limbs, in the vicinity of peripheral nerves

increased hemolymphoid system tumor incidence ( J:240370 )

• mice develop hematopoietic neoplasms, with 44% of mice exhibiting hematopoietic malignancies

• mice succumb to hematopoietic malignancies, with enlarged liver and/or spleen

• hematopoietic malignancies typically occur in 6-8 months old mice

nervous system

increased neurofibrosarcoma incidence ( J:240370 )

• malignant peripheral nerve sheath tumors are evident typically at 4-6 months of age on the shoulders, ribs, or limbs, in the vicinity of peripheral nerves

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
malignant peripheral nerve sheath tumor		DOID:5940		J:240370

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