Phenotypes Associated with This Genotype

Genotype
MGI:3511193

• Allelic Composition: Zic1/Zic4^tm1Kjmi/Zic1⁺
• Genetic Background: involves: 129P2/OlaHsd * C57BL/6J

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

mortality/aging

postnatal lethality, incomplete penetrance ( J:93955 )

• severely affected heterozygotes (15% of all heterozygotes) do not survive past weaning

behavior/neurological

impaired righting response ( J:93955 )

• severely affected heterozygotes (15% of all heterozygotes) do not develop a normal righting reflex by P10

ataxia ( J:93955 )

growth/size/body

decreased body weight ( J:93955 )

• average body weight in severely affected heterozygotes (15% of all heterozygotes) is 45% that of wild-type littermates

nervous system

abnormal cerebellar foliation ( J:93955 )

• in 85% of heterozygotes mild defects are seen in the anterior folia

• in 15% of heterozygotes severe foliar defects are seen

abnormal cerebellum vermis morphology ( J:93955 )

• in mildly affected heterozygotes, the vermis is slightly smaller compared to wild-type mice

cerebellum vermis hypoplasia ( J:93955 )

• in severely affected heterozygotes disproportionate vermis hypoplasia is seen

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Dandy-Walker syndrome		DOID:2785	OMIM:220200	J:93955