Phenotypes associated with this allele

• Allele Symbol: Tg(Camk2a-cre)2Szi
• Allele Name: transgene insertion 2, Scott Zeitlin
• Allele ID: MGI:2177769
• Summary: 8 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Lamc1^tm1Strl/Lamc1^tm1Strl Tg(Camk2a-cre)2Szi/0	involves: 129P2/OlaHsd * C57BL/6 * CBA	MGI:2683484
cn2	Htt^tm1Szi/Htt^tm2Szi Tg(Camk2a-cre)2Szi/0	involves: 129S/SvEv * 129S1/Sv * C57BL/6 * CBA	MGI:3605770
cn3	Hcn1^tm1Kndl/Hcn1^tm1Kndl Tg(Camk2a-cre)2Szi/?	involves: 129S/SvEv * C57BL/6 * CBA	MGI:2686969
cn4	Igf1r^tm1Arge/Igf1r^tm2Arge Tg(Camk2a-cre)2Szi/0	involves: 129S/SvEv * C57BL/6J * CBA	MGI:3038640
cn5	Ptpn11^tm1Gsf/Ptpn11^tm1Gsf Tg(Camk2a-cre)2Szi/0	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA	MGI:3522138
cn6	Hif1a^tm3Rsjo/Hif1a^tm3Rsjo Tg(Camk2a-cre)2Szi/0	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA	MGI:4418484
cn7	Hap1^tm1Id/Hap1^tm2Id Tg(Camk2a-cre)2Szi/0	involves: 129S1/Sv * C57BL/6 * CBA	MGI:3521638
cn8	Rapgef3^tm1.1Geno/Rapgef3^tm1.1Geno Tg(Camk2a-cre)2Szi/0	involves: 129S2/SvPas * C57BL/6J * CBA	MGI:5521492

Genotype
MGI:2683484

cn1

• Allelic Composition: Lamc1^tm1Strl/Lamc1^tm1Strl; Tg(Camk2a-cre)2Szi/0
• Genetic Background: involves: 129P2/OlaHsd * C57BL/6 * CBA

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

behavior/neurological

tremors ( J:86714 )

• tremor that persists even under deep anesthesia

forelimb paralysis ( J:86714 )

• in the most severely affected mutants, motor defects are also seen in the front legs

hindlimb paralysis ( J:86714 )

• mutants appear normal for 2-3 weeks after birth but by 4 weeks, begin to show hind leg weakness that leads to completely paralyzed hind legs by 3 months of age

nervous system

abnormal Schwann cell morphology ( J:86714 )

• Schwann cells are present in nerves but do not differentiate; they have a discontinuous basal lamina, are arrested at the premyelination stage and do not form myelin

abnormal axon radial sorting ( J:86714 )

• defect in axon sorting and myelination of the dorsal root of the spinal cord

• sciatic nerves are only partially myelinated and have large bundles of naked, unsorted axons

abnormal dorsal spinal root morphology ( J:86714 )

• defect in axon sorting and myelination of the dorsal root of the spinal cord

abnormal sciatic nerve morphology ( J:86714 )

• sciatic nerves are only partially myelinated and have large bundles of naked, unsorted axons

abnormal ventral spinal root morphology ( J:86714 )

• defect in axon sorting and myelination of the dorsal root of the spinal cord; more severe than in dorsal root

abnormal myelination ( J:86714 )

• Schwann cells in nerves have normal proliferation and apoptosis during development however, they fail to sort and myelinate axons

abnormal peripheral nervous system regeneration ( J:86714 )

• regeneration of the sciatic nerve after injury is impaired

homeostasis/metabolism

abnormal response to injury ( J:86714 )

• regeneration of the sciatic nerve after injury is impaired

muscle

muscular atrophy ( J:86714 )

• muscular atrophy is seen by 3 months of age

Genotype
MGI:3605770

cn2

• Allelic Composition: Htt^tm1Szi/Htt^tm2Szi; Tg(Camk2a-cre)2Szi/0
• Genetic Background: involves: 129S/SvEv * 129S1/Sv * C57BL/6 * CBA

mortality/aging

premature death ( J:65520 )

• mutants that are hydrocephalic die by P35

• none survive longer than 13 months

growth/size/body

decreased body size ( J:65520 )

• smaller than controls at weaning and at P60

behavior/neurological

limb grasping ( J:65520 )

• abnormal limb clasping at P21 that becomes progressively more severe such that mice curl their body upon clasping and maintain the posture for several seconds following return to cage

tremors ( J:65520 )

• exhibit a slight tremor at 10-12 months of age

impaired coordination ( J:65520 )

• exhibit motor defects in mutants subjected to cage-top rotation test and elevated wire rod hanging test

decreased locomotor activity ( J:65520 )

• noticeably hypoactive at 10-12 months of age

nervous system

hydrocephaly ( J:65520 )

• about 8% are hydrocephalic by P10

abnormal brain internal capsule morphology ( J:65520 )

abnormal striatum morphology ( J:65520 )

• striatum is disorganized at 8-months of age

abnormal cerebral cortex morphology ( J:65520 )

• cortex is disorganized at 8-months of age

gliosis ( J:65520 )

• gliosis is seen throughout the forebrain in young mutants

astrocytosis ( J:65520 )

• reactive astocytosis in the entorhinal cortex, striatum, and frontal cortex at 4 and 8 months of age

neurodegeneration ( J:65520 )

• neurodegeneration in the caudal/lateral region of the cortex at 4-6 months of age and in the rostral and caudal portions of the brain adjacent to the external capsule and in the entorhinal cortex at 8 months of age

• exhibit shrunken eosinophilic neurons, indicative of neuronal damage, in the hippocampus at 8-months of age

• 8 month old mutants exhibit neurodegeneration in the external capsule fibre tracts and in fibre bundles of the internal capsule within the striatum, in the amygdala and in both the frontal and dorsal cortex

• degeneration is essentially complete by 10 months

reproductive system

oligozoospermia ( J:65520 )

♂ • exhibit a reduction in the number of spermatocytes and round spermatids in the seminiferous tubules and in mature motile sperm in the lumen of the epididymis

abnormal seminiferous tubule morphology ( J:65520 )

♂ • disorganized and contain fewer spermatocytes and round spermatids compared to controls

small testis ( J:65520 )

♂ • testis is about 50% the weight of controls

reduced male fertility ( J:65520 )

♂

endocrine/exocrine glands

abnormal seminiferous tubule morphology ( J:65520 )

♂ • disorganized and contain fewer spermatocytes and round spermatids compared to controls

small testis ( J:65520 )

♂ • testis is about 50% the weight of controls

cellular

oligozoospermia ( J:65520 )

♂ • exhibit a reduction in the number of spermatocytes and round spermatids in the seminiferous tubules and in mature motile sperm in the lumen of the epididymis

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Huntington's disease		DOID:12858	OMIM:143100	J:65520

Genotype
MGI:2686969

cn3

• Allelic Composition: Hcn1^tm1Kndl/Hcn1^tm1Kndl; Tg(Camk2a-cre)2Szi/?
• Genetic Background: involves: 129S/SvEv * C57BL/6 * CBA

behavior/neurological

behavior/neurological phenotype ( J:86763 )

N • no learned motor skill deficit could be detected using rotorod tests

Genotype
MGI:3038640

cn4

• Allelic Composition: Igf1r^tm1Arge/Igf1r^tm2Arge; Tg(Camk2a-cre)2Szi/0
• Genetic Background: involves: 129S/SvEv * C57BL/6J * CBA

nervous system

decreased oligodendrocyte progenitor number ( J:88198 )

• oligodendrocyte progenitor cells fail to proliferate and an increase in apoptosis is seen in the middle region of the corpus callosum following cuprizone exposure

abnormal CNS glial cell morphology ( J:88198 )

• micoglia/macrophages persist in the middle region of the corpus callosum in mutants 9 weeks after cuprizone exposure but not in wild-types

abnormal oligodendrocyte morphology ( J:88198 )

• mature oligodendrocyte fail to regenerate in the middle region of the corpus callosum following cuprizone exposure

abnormal myelination ( J:88198 )

• 9 and 14 weeks after cuprizone induced demyelination, remyelination is absent in the middle region of the corpus callosum of the forebrain in mutants

• 9 and 14 weeks after cuprizone induced demyelination, remyelination has occurred in the extreme ventral and dorsal edges of the callosum in mutants and throughout the corpus callosum in wild-types

• prior to cuprizone treatment myelination is normal in mutants

cellular

decreased oligodendrocyte progenitor number ( J:88198 )

• oligodendrocyte progenitor cells fail to proliferate and an increase in apoptosis is seen in the middle region of the corpus callosum following cuprizone exposure

Genotype
MGI:3522138

cn5

• Allelic Composition: Ptpn11^tm1Gsf/Ptpn11^tm1Gsf; Tg(Camk2a-cre)2Szi/0
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA

adipose tissue

increased brown adipose tissue amount ( J:94738 )

• at 8 weeks of age, males had 9% more brown adipose tissue and females had 80% more than controls

increased white adipose tissue amount ( J:94738 )

• at 8 weeks of age, male mutants had 32% more white adipose tissue and females had 123% more than controls

endocrine/exocrine glands

hypersecretion of corticosterone ( J:94738 )

growth/size/body

obese ( J:94738 )

• at 8 weeks of age, males and females gained 28% and 21%, respectively, more weight than wild-types or heterozygotes, developing early-onset obesity

• male and female adults weighed ~30 to 50% more than wild-type

increased body length ( J:94738 )

• increased snout-anus length

increased susceptibility to weight gain ( J:94738 )

• male mutants gained 150% of body weight between P23-32 compared with controls while females gained 172% of body weight compared with controls despite similar or slightly reduced amounts of food consumption

enlarged liver ( J:94738 )

• liver size was increased by 12% in males, with a lesser extent of increase in females

homeostasis/metabolism

abnormal circulating hormone level ( J:94738 )

increased circulating corticosterone level ( J:94738 )

increased circulating insulin level ( J:94738 )

• mutants in both the fed and fasting states showed an increase in insulin levels

increased circulating leptin level ( J:94738 )

• 2.7-fold and 4.6-fold increase in serum leptin levels in male and female mutants, respectively

increased circulating growth hormone level ( J:94738 )

• hypersecretion of growth hormone was observed in male mutants and to a lesser extent in females

increased circulating thyroid-stimulating hormone level ( J:94738 )

• increased serum thyroid-stimulating hormone levels in 13-15 week old females

decreased circulating thyroxine level ( J:94738 )

• lower thyroxine levels in 13-15 week old females

decreased circulating triiodothyronine level ( J:94738 )

• modestly decreased serum triiodothyronine levels in 13-15 week old females

increased circulating triglyceride level ( J:94738 )

• 20% more serum triglyceride levels in both males and females

decreased body temperature ( J:94738 )

• significantly lower body temperature (35.8 versus 36.5C)

abnormal glucose homeostasis ( J:94738 )

hypoglycemia ( J:94738 )

• mutants fasted for 20 hours became hypoglycemic compared to controls

hyperglycemia ( J:94738 )

• hyperglycemic at 8 weeks of age when fed a normal diet

increased liver glycogen level ( J:94738 )

• elevated glycogen deposits in livers

increased liver triglyceride level ( J:94738 )

• abnormal accumulation of triglycerides in the liver

liver/biliary system

abnormal liver morphology ( J:94738 )

• abnormal accumulation of triglycerides and elevated glycogen deposits in the liver

enlarged liver ( J:94738 )

• liver size was increased by 12% in males, with a lesser extent of increase in females

increased liver glycogen level ( J:94738 )

• elevated glycogen deposits in livers

increased liver triglyceride level ( J:94738 )

• abnormal accumulation of triglycerides in the liver

hepatic steatosis ( J:94738 )

• fatty liver in 15-week old mutants

reproductive system

reduced female fertility ( J:94738 )

♀ • for 3 months, 8/12 mutant females delivered pups, and the average litter number was 45% of controls

Genotype
MGI:4418484

cn6

• Allelic Composition: Hif1a^tm3Rsjo/Hif1a^tm3Rsjo; Tg(Camk2a-cre)2Szi/0
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * CBA

mortality/aging

increased susceptibility to induced morbidity/mortality ( J:121971 )

• following transient middle cerebral artery occlusion

homeostasis/metabolism

abnormal physiological response to xenobiotic ( J:121971 )

• the neuroprotective effects on infarct size and neurological deficit scores of 3,4-dihydroxybenzoic acid, deferoxamine (DFO), and DP following transient middle cerebral artery occlusion is decreased compared to in similarly treated Hif1a^tm3Rsjo homozygotes

decreased susceptibility to ischemic brain injury ( J:98637 )

• following bilateral common carotid artery occlusion, mice exhibit decreased neuronal damage compared to in similarly treated Hif1a^tm3Rsjo homozygotes

increased susceptibility to ischemic brain injury ( J:121971 )

• following transient middle cerebral artery occlusion, mice exhibit increased infarct size, increased neurological deficit scores, and mortality compared with similarly treated Hif1a^tm3Rsjo homozygotes

increased cerebral infarct size ( J:121971 )

• 4 days after transient middle cerebral artery occlusion, infarct size is larger than in similarly treated Hif1a^tm3Rsjo homozygotes

• the neuroprotective effects on infarct size and neurological deficit scores of 3,4-dihydroxybenzoic acid, deferoxamine (DFO), and DP following transient middle cerebral artery occlusion is decreased compared to in similarly treated Hif1a^tm3Rsjo homozygotes

• however, mice still respond to neuroprotective hypoxia with reduced infarct volume at shorter exposure periods than wild-type mice

nervous system

decreased susceptibility to ischemic brain injury ( J:98637 )

• following bilateral common carotid artery occlusion, mice exhibit decreased neuronal damage compared to in similarly treated Hif1a^tm3Rsjo homozygotes

increased susceptibility to ischemic brain injury ( J:121971 )

• following transient middle cerebral artery occlusion, mice exhibit increased infarct size, increased neurological deficit scores, and mortality compared with similarly treated Hif1a^tm3Rsjo homozygotes

increased cerebral infarct size ( J:121971 )

• 4 days after transient middle cerebral artery occlusion, infarct size is larger than in similarly treated Hif1a^tm3Rsjo homozygotes

• the neuroprotective effects on infarct size and neurological deficit scores of 3,4-dihydroxybenzoic acid, deferoxamine (DFO), and DP following transient middle cerebral artery occlusion is decreased compared to in similarly treated Hif1a^tm3Rsjo homozygotes

• however, mice still respond to neuroprotective hypoxia with reduced infarct volume at shorter exposure periods than wild-type mice

hydrocephaly ( J:121971 )

• uncommon

behavior/neurological

abnormal behavior ( J:121971 )

• following transient middle cerebral artery occlusion, mice exhibit increased neurological deficit scores compared with similarly treated Hif1a^tm3Rsjo homozygotes

• the neuroprotective effects on infarct size and neurological deficit scores of 3,4-dihydroxybenzoic acid, deferoxamine (DFO), and DP following transient middle cerebral artery occlusion is decreased compared to in similarly treated Hif1a^tm3Rsjo homozygotes

Genotype
MGI:3521638

cn7

• Allelic Composition: Hap1^tm1Id/Hap1^tm2Id; Tg(Camk2a-cre)2Szi/0
• Genetic Background: involves: 129S1/Sv * C57BL/6 * CBA

growth/size/body

postnatal growth retardation ( J:94581 )

• postnatal lethality is no longer seen in mutants however a slight growth deficiency is seen with mutants at P21 only about 70% of the size of wild-type littermates

Genotype
MGI:5521492

cn8

• Allelic Composition: Rapgef3^tm1.1Geno/Rapgef3^tm1.1Geno; Tg(Camk2a-cre)2Szi/0
• Genetic Background: involves: 129S2/SvPas * C57BL/6J * CBA

nervous system

nervous system phenotype ( J:182701 )

N • mice exhibit normal CA1 pyramidal spine density and synaptic transmission