A defect in a novel nek-family kinase causes cystic kidney disease in the mouse and in zebrafish
2002
The murine autosomal recessive juvenile cystic kidney ( jck )
mutation results in polycystic kidney disease. We have identified in
jck mice a mutation in Nek8 , a novel and highly conserved
member of the Nek kinase family. In vitro expression of mutated Nek8
results in enlarged, multinucleated cells with an abnormal actin cytoskeleton.
To confirm that a defect in the Nek8 gene can cause cystic disease,
we performed a cross-species analysis: injection of zebrafish embryos with a
morpholino anti-sense oligonucleotide corresponding to the ortholog of
Nek8 resulted in the formation of pronephric cysts. These results
demonstrate that comparative analysis of gene function in different model
systems represents a powerful means to annotate gene function.
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