Mouse Genome Informatics
cn
    Gt(ROSA)26Sortm1(HD*103Q)Xwy/?
Tg(Nes-cre)1Kln/?

involves: 129S1/Sv * 129X1/SvJ * C57BL/6 * SJL
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
nervous system
• 35 of 37 neurons display dysmorphic axons compared to only 5 of 40 wild-type axons in cortex in 6-month old mice
• at 1 year of age, an increase in degenerating neurons, including pyramidal neurons, is seen in the cortex compared to wild-type mice
• 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
• at 1 year of age, an increase in degenerating neurons, including pyramidal neurons, is seen in the cortex compared to wild-type mice
• spontaneous inhibitory postsynaptic current frequency but not amplitude is significantly decreased; however no change in spontaneous excitatory postsynaptic currents is seen

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

Mouse Models of Human Disease
OMIM IDRef(s)
Huntington Disease; HD 143100 J:99759