ZFIN ID: ZDB-PUB-120628-4
The ciliary protein Nek8/Nphp9 acts downstream of Inv/Nphp2 during pronephros morphogenesis and left-right establishment in zebrafish
Fukui, H., Shiba, D., Asakawa, K., Kawakami, K., and Yokoyama, T.
Date: 2012
Source: FEBS letters   586(16): 2273-2279 (Journal)
Registered Authors: Kawakami, Koichi
Keywords: primary cells, inv, nek8, zebrafish, nephronophthisis
MeSH Terms:
  • Animals
  • Body Patterning
  • Cilia/metabolism*
  • Cloning, Molecular
  • Gene Expression Profiling
  • Gene Expression Regulation, Developmental*
  • Glutathione Transferase/metabolism
  • Green Fluorescent Proteins/metabolism
  • In Situ Hybridization
  • Kidney/cytology
  • Mice
  • Models, Genetic
  • Phenotype
  • Protein Kinases/metabolism*
  • Protein-Serine-Threonine Kinases/metabolism*
  • Time Factors
  • Transcription Factors/metabolism*
  • Transgenes
  • Zebrafish
  • Zebrafish Proteins/metabolism*
PubMed: 22687244 Full text @ FEBS Lett.

Nephronophthisis (NPHP) is an autosomal recessive cystic kidney disease. Among 12 reported Nphp gene products, Inv/Nphp2, Nphp3 and Nek8/Nphp9 are localized to the proximal segment in the primary cilium. However, the functional relationships are unknown. This study focused on phenotype analysis of nek8 knockdown embryos and the genetic relationship between nek8 and inv in zebrafish. Knockdown of nek8 produced both pronephric cysts and abnormal cardiac looping. Simultaneous knockdown of nek8 and inv synergistically increased the incidence of these defects. Interestingly, nek8 mRNA rescued inv morphant phenotypes, although inv mRNA could not rescue nek8 morphant phenotypes. These results suggest that Nek8 acts downstream of Inv function.