Mouse Genome Informatics
tg
    Tg(JPH3*)CXwy/0
involves: FVB/N
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
nervous system
• low levels of ubiquitin immunoreactive inclusion bodies are detected at 12 months of age
• prominent ubiquitin immunoreactive inclusion bodies are detected at 12 months of age
• prominent ubiquitin immunoreactive inclusion bodies are detected at 12 months of age
• reduction in cortical but not striatal volume with age
• prominent ubiquitin immunoreactive inclusion bodies are detected in the upper cortical layers at 12 months of age
• CUG RNA foci are detected in the cortex
• prominent ubiquitin immunoreactive inclusion bodies are detected at 12 months of age
• inclusions are most abundant in the upper cortical layers, hippocampus, and amygdala with relatively low levels in the deep cortical layers and striatum
• inclusions can be detected as early as 3 months of age in the cortex and hippocampus
• selective forebrain atrophy develops with age

behavior/neurological
• progressive motor deficits in a rotarod assay at 6 and 12 months of age, but not at 3 months of age

Mouse Models of Human Disease
OMIM IDRef(s)
Huntington Disease-Like 2; HDL2 606438 J:174267