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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID 		Tg(CMV-ATXN3*94Q)94Pama
transgene insertion 94, Patricia Maciel
MGI:4822570
Summary 2 genotypes


Genotype
MGI:4822572
tg1
Allelic
Composition		 Tg(CMV-ATXN3*94Q)94Pama/Tg(CMV-ATXN3*94Q)94Pama
Genetic
Background		 B6.FVB-Tg(CMV-ATXN3*94Q)94Pama
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
behavior/neurological
behavior/neurological phenotype ( J:163032 )
N
• mice exhibit normal gait pattern and limb strength between 16 and 84 weeks of age
impaired coordination ( J:163032 )
• beginning at 16 weeks on a rotarod
• homozygous mice tend to spend less time on a rotarod than heterozygous mice
decreased locomotor activity ( J:163032 )
• mice exhibit a decrease of locomotor activity compared with wild-type mice

nervous system
astrocytosis ( J:163032 )
• at 49 weeks in the substantia nigra and vestibular nuclei
neurodegeneration ( J:163032 )
• at 16 weeks, mice exhibit mild neuronal atrophy in areas such as the pontine and dentate nuclei unlike wild-type mice

Mouse Models of Human Disease
 DO ID OMIM ID(s) Ref(s)
Machado-Joseph disease DOID:1440 OMIM:109150
 J:163032




Genotype
MGI:4822571
tg2
Allelic
Composition		 Tg(CMV-ATXN3*94Q)94Pama/0
Genetic
Background		 B6.FVB-Tg(CMV-ATXN3*94Q)94Pama
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
behavior/neurological
behavior/neurological phenotype ( J:163032 )
N
• mice exhibit normal gait pattern and limb strength between 16 and 84 weeks of age
impaired coordination ( J:163032 )
• beginning at 16 weeks on a rotarod
• heterozygous mice tend to spend more time on a rotarod than homozygous mice
decreased locomotor activity ( J:163032 )
• mice tend to exhibit a decrease of locomotor activity compared with wild-type mice

nervous system
nervous system phenotype ( J:163032 )
N
• mice do not exhibit intranuclear inclusions at 49 weeks
astrocytosis ( J:163032 )
• at 49 weeks in the substantia nigra and vestibular nuclei
neurodegeneration ( J:163032 )
• at 16 weeks, mice exhibit mild neuronal atrophy unlike wild-type mice

cellular
abnormal cell physiology ( J:163032 )
• mice exhibit generational instability and somatic mosaicism in CAG repeats of the transgene

Mouse Models of Human Disease
 DO ID OMIM ID(s) Ref(s)
Machado-Joseph disease DOID:1440 OMIM:109150
 J:163032




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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Send questions and comments to User Support. 		last database update
04/30/2024
MGI 6.23 		The Jackson Laboratory