Phenotypes Associated with This Genotype

Genotype
MGI:5806875

• Allelic Composition: Tg(Venus/SOX10*)55Kein/Tg(Venus/SOX10*)55Kein
• Genetic Background: involves: C3H/He * C57BL/6

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

postnatal lethality ( J:227442 )

• mice with 4 or 5 copies of the transgene die by P4

nervous system

abnormal oligodendrocyte morphology ( J:227442 )

• terminal differentiation of oligodendrocyte lineage cells in the developing spinal cord and brain is delayed in postnatal mice with 5 copies of the transgene

abnormal enteric nervous system morphology ( J:227442 )

• 100% of mice with 4 or 5 copies of the transgene show defects of the nerve plexus, namely hypoganglionosis in the distal part of the large intestine

• hypoganglionosis expands over the entire large intestine in mice with 5 copies of the transgene

digestive/alimentary system

abnormal large intestine morphology ( J:227442 )

• inflation of the large intestine is seen in dead mice with 4 or 5 copies of the transgene

cellular

abnormal oligodendrocyte morphology ( J:227442 )

• terminal differentiation of oligodendrocyte lineage cells in the developing spinal cord and brain is delayed in postnatal mice with 5 copies of the transgene

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