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Phenotypes Associated with This Genotype
Genotype
MGI:3838165
Allelic
Composition
Tg(ATXN3*)67.2Cce/0
Tg(ATXN3*)84.2Cce/0
Genetic
Background
involves: C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(ATXN3*)67.2Cce mutation (0 available)
Tg(ATXN3*)84.2Cce mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
growth/size/body
• mice have slower weight gain with a 28% weight reduction by 48 weeks of age compared to controls
• mice weigh 20% less than controls at weaning and 25% less at 20 weeks of age
• mice lose weight after 32 weeks of age

behavior/neurological
• almost all mice show excessive grooming
• some mice clasp fore- and hind-limbs when lifted by the tail by 16 weeks of age
• mice have mild tremors
• mice have a prominent wide gait and a lowered pelvis during grid climbing
• mice are less active and inquisitive than controls
• mice have no negative geotaxis after 12 weeks of age

nervous system
• mice have marked gliosis in areas of neuronal loss
• neuronal degeneration is evident in the cerebellum
• 20% of Purkinje cells suffer from dendrite atrophy
• is observed in >50% of pontine nuclei and >50% of dentate nuclei
• mice have occasional axonal abnormalities and even rarer axonal degeneration
• mice have a minor degree of de- and re- myelination in the sciatic nerves by 6 month of age
• by 14 months of age, extensive myelin abnormalities exist including demyelinated and thinly myelinated axons, and "onion bulb" formations

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
Machado-Joseph disease DOID:1440 OMIM:109150
J:76495


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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last database update
11/12/2024
MGI 6.24
The Jackson Laboratory