Phenotypes associated with this allele

• Allele Symbol: Gt(ROSA)26Sor^{tm1(HD*103Q)Xwy}
• Allele Name: targeted mutation 1, X William Yang
• Allele ID: MGI:3583991
• Summary: 2 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Emx1^tm1(cre)Ito/? Gt(ROSA)26Sor^tm1(HD*103Q)Xwy/?	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL	MGI:3584454
cn2	Gt(ROSA)26Sor^tm1(HD*103Q)Xwy/? Tg(Nes-cre)1Kln/?	involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL	MGI:3584455

Genotype
MGI:3584454

cn1

• Allelic Composition: Emx1^tm1(cre)Ito/?; Gt(ROSA)26Sor^{tm1(HD*103Q)Xwy}/?
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL

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

nervous system

nervous system phenotype ( J:99759 )

N • mice have comparable locomotor activity to wild-type controls

N • gliosis levels are comparable to wild-type

N • dendrites with abnormal or wavy morphology occur with similar frequency in mutants and wild-type controls

N • very few neurons showing early stages of degeneration are observed

N • electrophysiology such as sIPSCs from cortical neurons are normal relative to controls

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
NOT	Huntington's disease	DOID:12858	OMIM:143100	J:99759

Genotype
MGI:3584455

cn2

• Allelic Composition: Gt(ROSA)26Sor^{tm1(HD*103Q)Xwy}/?; Tg(Nes-cre)1Kln/?
• Genetic Background: involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL

nervous system

abnormal cerebral cortex pyramidal cell morphology ( J:99759 )

• 35 of 37 neurons display dysmorphic axons compared to only 5 of 40 wild-type axons in cortex in 6-month old mice

cerebral cortex pyramidal cell degeneration ( J:99759 )

• at 1 year of age, an increase in degenerating neurons, including pyramidal neurons, is seen in the cortex compared to wild-type mice

gliosis ( J:99759 )

• the density of GFAP+ cells is increased 9-fold and 6-fold in the cortex and striatum, respectively, compared to wild-type littermates at 6 months

neurodegeneration ( J:99759 )

• at 1 year of age, an increase in degenerating neurons, including pyramidal neurons, is seen in the cortex compared to wild-type mice

abnormal inhibitory postsynaptic currents ( J:99759 )

• spontaneous inhibitory postsynaptic current frequency but not amplitude is significantly decreased; however no change in spontaneous excitatory postsynaptic currents is seen

behavior/neurological

decreased locomotor activity ( J:99759 )

• activity is reduced at 6, 8, and 10 months of age but not at 2 months of age

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Huntington's disease		DOID:12858	OMIM:143100	J:99759