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PMID: 11062270 Published · ppublish English Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S.

Chlamydomonas IFT88 and its mouse homologue, polycystic kidney disease gene tg737, are required for assembly of cilia and flagella.

The Journal of cell biology ·Vol. 151 ·No. 3 ·2000-10-30 ·Pages 709-18

Pazour GJ, Dickert BL, Vucica Y, Seeley ES, Rosenbaum JL, Witman GB, Cole DG

Abstract

Intraflagellar transport (IFT) is a rapid movement of multi-subunit protein particles along flagellar microtubules and is required for assembly and maintenance of eukaryotic flagella. We cloned and sequenced a Chlamydomonas cDNA encoding the IFT88 subunit of the IFT particle and identified a Chlamydomonas insertional mutant that is missing this gene. The phenotype of this mutant is normal except for the complete absence of flagella. IFT88 is homologous to mouse and human genes called Tg737. Mice with defects in Tg737 die shortly after birth from polycystic kidney disease. We show that the primary cilia in the kidney of Tg737 mutant mice are shorter than normal. This indicates that IFT is important for primary cilia assembly in mammals. It is likely that primary cilia have an important function in the kidney and that defects in their assembly can lead to polycystic kidney disease.

MeSH Terms
Amino Acid Sequence Animals Chlamydomonas/cytology,genetics Cilia/genetics,metabolism,pathology,ultrastructure Cloning, Molecular Conserved Sequence Flagella/genetics,metabolism,pathology,ultrastructure Humans Kidney/metabolism,pathology Meiosis Mice Mice, Knockout Microscopy, Electron, Scanning Molecular Motor Proteins/genetics,metabolism,pathology,ultrastructure Molecular Sequence Data Mutation/genetics Phenotype Plant Proteins Polycystic Kidney, Autosomal Recessive/genetics,pathology,physiopathology Protein Binding Protein Subunits Proteins/chemistry,genetics Protozoan Proteins/chemistry,genetics,metabolism Repetitive Sequences, Amino Acid/genetics,physiology Sequence Alignment Sequence Homology, Amino Acid Tumor Suppressor Proteins
Chemicals
IFT88 protein, Chlamydomonas IFT88 protein, human Molecular Motor Proteins Plant Proteins Protein Subunits Proteins Protozoan Proteins Tg737Rpw protein, mouse Tumor Suppressor Proteins
Authors & Affiliations
7 authors, click to expand affiliations / ORCID
Pazour G J
Department of Cell Biology, University of Massachusetts Medical School, Worcester, Massachusetts 01655, USA.
Dickert B L
Vucica Y
Seeley E S
Rosenbaum J L
Witman G B
Cole D G
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Article Info
Journal
The Journal of cell biology
Abbr.
J Cell Biol
ISSN
0021-9525
DOI
10.1083/jcb.151.3.709
Published
2000-10-30
Pages
709-18
Language
English
Region
United States
NLM ID
0375356
PMCID
PMC2185580
Subset
IM
Grants
NIGMS NIH HHS · GM30626 · United States
NIGMS NIH HHS · GM14642 · United States
External Links
PubMed source DOI: 10.1083/jcb.151.3.709 PMC Full Text View journal details
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