Phenotypes Associated with This Genotype

Genotype
MGI:7541371

• Allelic Composition: Wls^em2Jgg/Wls^em2Jgg
• Genetic Background: involves: C57BL/6J

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

mortality/aging

perinatal lethality, complete penetrance ( J:341542 )

cellular

abnormal neuron differentiation ( J:341542 )

• embryonic mice show a reduction of neurogenesis in the brain associated with a defective cell cycle of intermediate neural progenitors, which stall at the S and G2/M phases

embryo

abnormal neural tube closure ( J:341542 )

• mice show defects in neural tube closure, with severely reduced or absent tail buds

abnormal tail bud morphology ( J:341542 )

• mice exhibit severely reduced or absent tail buds

• dams administered the Wnt agonist CHIR99021 intraperitoneally twice daily starting at E4.5 and until E12.5, show near-complete rescue of tail growth in embryos

• dams administered CHIR99021 intraperitoneally twice daily starting at E4.5 and until E18.5, have embryos with increased tail length

limbs/digits/tail

abnormal tail bud morphology ( J:341542 )

• mice exhibit severely reduced or absent tail buds

• dams administered the Wnt agonist CHIR99021 intraperitoneally twice daily starting at E4.5 and until E12.5, show near-complete rescue of tail growth in embryos

• dams administered CHIR99021 intraperitoneally twice daily starting at E4.5 and until E18.5, have embryos with increased tail length

adactyly ( J:341542 )

• mice show reduced numbers of forelimb and hindlimb digits

decreased caudal vertebrae number ( J:341542 )

• mice exhibit reduced numbers of caudal vertebrae

• dams administered CHIR99021 intraperitoneally twice daily starting at E4.5 and until E18.5, have embryos with increased number of caudal vertebrae

nervous system

decreased brain size ( J:341542 )

abnormal dentate gyrus morphology ( J:341542 )

• mice show severely defective hippocampal dentate gyrus

abnormal nervous system development ( J:341542 )

• mice show cobblestone-like penetration of neurons through the disrupted pial basement membrane

abnormal neuron differentiation ( J:341542 )

• embryonic mice show a reduction of neurogenesis in the brain associated with a defective cell cycle of intermediate neural progenitors, which stall at the S and G2/M phases

abnormal neural tube closure ( J:341542 )

• mice show defects in neural tube closure, with severely reduced or absent tail buds

decreased neuron number ( J:341542 )

• mice show decreased number of cortical neurons

renal/urinary system

hydronephrosis ( J:341542 )

• cystic medullary hydronephrosis

• embryos from dams administered CHIR99021 intraperitoneally twice daily starting at E4.5 and until E18.5 show a reduction in incidence of hydronephrosis from 75% to 22.7%

skeleton

decreased caudal vertebrae number ( J:341542 )

• mice exhibit reduced numbers of caudal vertebrae

• dams administered CHIR99021 intraperitoneally twice daily starting at E4.5 and until E18.5, have embryos with increased number of caudal vertebrae

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Zaki syndrome		DOID:0070473	OMIM:619648	J:341542