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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(ATXN3*)67.2Cce
transgene insertion 67.2, Cemal K Cemal
MGI:3838156
Summary 2 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cx1
Tg(ATXN3*)67.2Cce/0
Tg(ATXN3*)84.2Cce/0
involves: C57BL/6 * CBA MGI:3838165
tg2
Tg(ATXN3*)67.2Cce/0 involves: C57BL/6 * CBA MGI:3838164


Genotype
MGI:3838165
cx1
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




Genotype
MGI:3838164
tg2
Allelic
Composition
Tg(ATXN3*)67.2Cce/0
Genetic
Background
involves: C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
growth/size/body
• mice have slower weight gain with a 8% weight reduction by 48 weeks of age compared to controls

behavior/neurological
• mice clasp fore- and hind-limbs when lifted by the tail
• mice have mild tremors
• mice have a wide gait and a lowered pelvis during grid climbing
• mice are mildly less active than controls starting at 4 weeks of age

nervous system
• Purkinje cell loss is evident
• mice have mild gliosis in areas of neuronal loss
• is observed in 30% of pontine nuclei and 20% of dentate nuclei

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





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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
11/19/2024
MGI 6.24
The Jackson Laboratory