Phenotypes Associated with This Genotype

Genotype
MGI:5052366

• Allelic Composition: Tg(JPH3*)CXwy/0
• Genetic Background: involves: FVB/N

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

nervous system

abnormal forebrain morphology ( J:174267 )

abnormal striatum morphology ( J:174267 )

• low levels of ubiquitin immunoreactive inclusion bodies are detected at 12 months of age

abnormal amygdala morphology ( J:174267 )

• prominent ubiquitin immunoreactive inclusion bodies are detected at 12 months of age

abnormal hippocampus morphology ( J:174267 )

• prominent ubiquitin immunoreactive inclusion bodies are detected at 12 months of age

abnormal cerebral cortex morphology ( J:174267 )

• 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

neuronal intranuclear inclusions ( J:174267 )

• 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

neurodegeneration ( J:174267 )

• selective forebrain atrophy develops with age

behavior/neurological

impaired coordination ( J:174267 )

• 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		DO ID	OMIM ID(s)	Ref(s)
Huntington's disease-like 2		DOID:0090104	OMIM:606438	J:174267