Department of Cell Biology; Program in Molecular Medicine
Amino Acid Sequence; Animals; Chlamydomonas; Cilia; Cloning, Molecular; Conserved Sequence; Flagella; Humans; Kidney; Meiosis; Mice; Mice, Knockout; Microscopy, Electron, Scanning; Molecular Motor Proteins; Molecular Sequence Data; Mutation; Phenotype; Polycystic Kidney, Autosomal Recessive; Protein Binding; Protein Subunits; Proteins; Protozoan Proteins; Repetitive Sequences, Amino Acid; Sequence Alignment; Sequence Homology, Amino Acid; *Tumor Suppressor Proteins
Cell Biology | Physiology
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.
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Citation: J Cell Biol. 2000 Oct 30;151(3):709-18.