title: An autosomal recessive polycystic kidney disease gene homolog is involved in intraflagellar transport in C. elegans ciliated sensory neurons.
abstract: In this report, we show that the Caenorhabditis elegans gene osm-5 is homologous to the Chlamydomonas gene IFT88 and the mouse autosomal recessive polycystic kidney disease (ARPKD) gene, Tg737. The function of this ARPKD gene may be evolutionarily conserved: mutations result in defective ciliogenesis in worms [1], algae [2], and mice [2, 3]. Intraflagellar transport (IFT) is essential for the development and maintenance of motile and sensory cilia [4]. The biochemically isolated IFT particle from Chlamydomonas flagella is composed of 16 polypeptides in one of two Complexes (A and B) [5, 6] whose movement is powered by kinesin II (anterograde) and cytoplasmic dynein (retrograde) [7-9]. We demonstrate that OSM-5 (a Complex B polypeptide), DAF-10 and CHE-11 (two Complex A polypeptides), and CHE-2 [10], a previously uncategorized IFT polypeptide, all move at the same rate in C. elegans sensory cilia. In the absence of osm-5, the C. elegans autosomal dominant PKD (ADPKD) gene products [11] accumulate in stunted cilia, suggesting that abnormal or lack of cilia or defects in IFT may result in diseases such as polycystic kidney disease (PKD).
authors: Department of Molecular, Cellular, and Developmental Biology, Yale University, New Haven, CT 06520, USA.
authors: H
authors: H
authors: Qin
authors: J L
authors: JL
authors: Rosenbaum
authors: M M
authors: MM
authors: Barr
mesh_terms: D000818:Animals; D017173:Caenorhabditis elegans / Q000235:genetics* / Q000378:metabolism; D002696:Chlamydomonas; D005407:Flagella / Q000378:metabolism; D020869:Gene Expression Profiling; D017238:Genes, Helminth / Q000502:physiology*; D051379:Mice; D009475:Neurons, Afferent / Q000378:metabolism; D010940:Plant Proteins; D017044:Polycystic Kidney, Autosomal Recessive / Q000235:genetics*; D011506:Proteins / Q000235:genetics*; D015800:Protozoan Proteins / Q000235:genetics; D025521:Tumor Suppressor Proteins
chemical_list: C416363:IFT88 protein, Chlamydomonas; D010940:Plant Proteins; D011506:Proteins; D015800:Protozoan Proteins; C490898:Tg737Rpw protein, mouse; D025521:Tumor Suppressor Proteins