Mouse Genome Informatics
cn
    Htttm1Szi/Htttm2Szi
Tg(Camk2a-cre)1Szi/0

involves: 129S/SvEv * 129S1/Sv * C57BL/6 * CBA
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• die between 11 and 13 months of age

growth/size
• smaller than controls at weaning and at P60

behavior/neurological
• abnormal limb clasping at P60 that becomes progressively more severe such that mice curl their body upon clasping and maintain the posture for several seconds following return to cage
• exhibit a slight tremor at 10-12 months of age
• exhibit motor defects in mutants subjected to cage-top rotation test and elevated wire rod hanging test
• noticeably hypoactive at 10-12 months of age

nervous system
• gliosis is seen throughout the forebrain in older mutants
• reactive astocytosis in the entorhinal cortex, striatum, and frontal cortex at 4 and 10 months of age
• 4 and 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

reproductive system
• disorganized and contain fewer spermatocytes and round spermatids compared to controls (J:65520)
• testis is about 50% the weight of controls (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 (J:65520)

endocrine/exocrine glands
• disorganized and contain fewer spermatocytes and round spermatids compared to controls (J:65520)
• testis is about 50% the weight of controls (J:65520)

Mouse Models of Human Disease
OMIM IDRef(s)
Huntington Disease; HD 143100 J:65520