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Phenotypes Associated with This Genotype
Genotype
MGI:5052366
Allelic
Composition
Tg(JPH3*)CXwy/0
Genetic
Background
involves: FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(JPH3*)CXwy mutation (1 available)
phenotype observed in females
phenotype observed in males
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 ID Ref(s)
Huntington Disease-Like 2; HDL2 606438 J:174267


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
07/19/2016
MGI 6.04
The Jackson Laboratory