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Phenotypes Associated with This Genotype
Genotype
MGI:5806872
Allelic
Composition
Tg(Venus/SOX10*)55Kein/0
Genetic
Background
involves: C3H/He * C57BL/6
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No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• a small proportion of mice with 3 copies of the transgene die around weaning

nervous system
• 4% of mice with 2 copies and 14% of mice with 3 copies of the transgene exhibit defects of the nerve plexus, namely hypoganglionosis in the distal part of the large intestine
• mice with 3 copies of the transgene show less refined Remak bundles at 6 weeks of age
• some Remak bundles are enlarged and the space between axons is occasionally narrower in mice with 3 copies of the transgene
• the median number of axons in a Remak bundle is larger in mice with 3 copies of the transgene, indicating that Remak bundles contain more axons per bundle
• mice with 3 copies of the transgene show less refined compact myelin sheaths in the sciatic nerve at 6 weeks of age
• persistence of a thinner myelin in the sciatic nerve of mice with 3 copies of the transgene

pigmentation
• 58% of mice with 2 copies and 81% with 3 copies of the transgene exhibit hypopigmentation of hair color in the forelock and the trunk; this is not seen in mice with one transgene copy

behavior/neurological
• 73% of mice with 2 copies and 35% mice with 3 copies of the transgene exhibit rotating behavior

digestive/alimentary system
• inflation of the large intestine is seen in dead mice with 3 copies of the transgene

hearing/vestibular/ear
• auditory threshold is higher in mice with 2 or 3 copies of the transgene, indicating sensory deafness
• mice with 2 or 3 copies of the transgene exhibit sensory deafness

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
PCWH syndrome DOID:0090111 OMIM:609136
J:227442


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last database update
03/19/2024
MGI 6.23
The Jackson Laboratory