PUBLICATION

A defect in a novel Nek-family kinase causes cystic kidney disease in the mouse and in zebrafish

Authors
Liu, S., Lu, W., Obara, T., Kuida, S., Lehoczky, J., Dewar, K., Drummond, I.A., and Beier, D.R.
ID
ZDB-PUB-021112-4
Date
2002
Source
Development (Cambridge, England)   129(24): 5839-5846 (Journal)
Registered Authors
Beier, David R., Drummond, Iain, Obara, Tomoko
Keywords
PKD, mouse models, zebrafish, Nek kinase
MeSH Terms
  • Amino Acid Sequence
  • Animals
  • COS Cells
  • Cell Nucleus/metabolism
  • Cloning, Molecular
  • Databases as Topic
  • Disease Models, Animal
  • Genome
  • Kidney Diseases, Cystic/genetics*
  • Mice
  • Mice, Inbred C57BL
  • Models, Genetic
  • Molecular Sequence Data
  • Mutation
  • Oligonucleotides, Antisense/pharmacology
  • Physical Chromosome Mapping
  • Protein Kinases*
  • Protein Serine-Threonine Kinases/genetics*
  • Protein Serine-Threonine Kinases/physiology*
  • RNA, Messenger/metabolism
  • Reverse Transcriptase Polymerase Chain Reaction
  • Time Factors
  • Zebrafish
  • Zebrafish Proteins
PubMed
12421721 Full text @ Development
Abstract
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.
Genes / Markers
Figures
Show all Figures
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Antibodies
Orthology
Engineered Foreign Genes
Mapping