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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Prnp-ATXN3)CVcg
transgene insertion C, Veronica Colomer Gould
MGI:3798044
Summary 2 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
tg1
Tg(Prnp-ATXN3)CVcg/Tg(Prnp-ATXN3)CVcg involves: C3H/HeJ * C57BL/6 MGI:3804855
tg2
Tg(Prnp-ATXN3)CVcg/0 involves: C3H/HeJ * C57BL/6 MGI:3804854


Genotype
MGI:3804855
tg1
Allelic
Composition
Tg(Prnp-ATXN3)CVcg/Tg(Prnp-ATXN3)CVcg
Genetic
Background
involves: C3H/HeJ * C57BL/6
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
• body weight progressively decreases, with weight only 40-60% of controls at moribund stage

behavior/neurological
• excessive grooming is observed at 2-3 months
• progressively deteriorating from 2.5 months
• at 2-3 months, mice display transiently clutched paws; at 3-5 months, paws are permanently clenched
• at 2-3 months of age, mice develop tremor; more pronounced at 4-8 months
• at 2-3 months, mice exhibit lateral body displacement while climbing, and display transiently uncoordinated limb extension (much more prolonged at 3-5 months)
• mice can not remain on rotating rod, starting at 1.75 months
• grip strength in fore- and hindlimbs is progressively impaired from 1.75 months of age
• mice develop progressively deteriorating gait from 2-3 months of age (gait is wide-based initially, wide-based and dragged at late stage, and scribbled at later to moribund stages)
• open-field activity is lower and progressively decreases from 1.75 months
• occasional seizures displayed at 3-5 months

nervous system
• occasional seizures displayed at 3-5 months
• total number of tyrosine hydroxylase-positive neurons in substantia nigra is 38% lower than in controls
• these are prominent (larger and more abundant) in neurons in selected brain regions and spinal cord compared to wild-type controls a 3-4 months worsening with age; in spinal cord, inclusions are observed in motor and sensory neurons

reproductive system

homeostasis/metabolism
• serum concentration is significantly lower in males than in wild-type males

skeleton
• starts to develop at 2-3months; at 3-5 months, mice show pronounced hunchback with low pelvic elevation and muscle wasting

endocrine/exocrine glands




Genotype
MGI:3804854
tg2
Allelic
Composition
Tg(Prnp-ATXN3)CVcg/0
Genetic
Background
involves: C3H/HeJ * C57BL/6
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
nervous system
• at 13 months, mice exhibit nucleolus-sized inclusions in neurons of deep cerebellar nuclei, pontine nuclei, and in spinal cord

homeostasis/metabolism

reproductive system
N
• testes are normal





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last database update
11/12/2024
MGI 6.24
The Jackson Laboratory