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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Prnp-ATXN3)BVcg
transgene insertion B, Veronica Colomer Gould
MGI:3798042
Summary 2 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
tg1
Tg(Prnp-ATXN3)BVcg/Tg(Prnp-ATXN3)BVcg involves: C3H/HeJ * C57BL/6 MGI:3804851
tg2
Tg(Prnp-ATXN3)BVcg/0 involves: C3H/HeJ * C57BL/6 MGI:3804850


Genotype
MGI:3804851
tg1
Allelic
Composition
Tg(Prnp-ATXN3)BVcg/Tg(Prnp-ATXN3)BVcg
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 2-4 months
• progressively deteriorating from 2.5 months
• at 2-4 months, mice display transiently clutched paws; at 4-8 months, paws are permanently clenched
• at 2-4 months of age, mice develop tremor; more pronounced at 4-8 months
• at 2-4 months, mice exhibit lateral body displacement while climbing, and display transiently uncoordinated limb extension (much more prolonged at 4-8 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 2-4 months of age (gait is wide-based initially, wide-based and dragged at late stage, and scribbled at later to moribund stages)
• at 2-4 months, mice exhibit lateral body displacement while climbing, and display transiently uncoordinated limb extension (much more prolonged at 4-8 months)
• occasional seizures displayed at 4-8 months

nervous system
• occasional seizures displayed at 4-8 months
• 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

skeleton
• starts to develop at 2-4 months; at 4-8 months, mice show pronounced hunchback with low pelvic elevation and muscle wasting

homeostasis/metabolism
• serum concentration is significantly lower in males than in wild-type males
• serum concentration is significantly lower in males than in wild-type males
• significantly lower in transgenic females compared to wild-type females
• significantly lower than in wild-type males

endocrine/exocrine glands

cellular




Genotype
MGI:3804850
tg2
Allelic
Composition
Tg(Prnp-ATXN3)BVcg/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
reproductive system
N
• males have normal testis morphology

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





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