An autosomal recessive polycystic kidney disease gene homolog is involved in intraflagellar transport in C. elegans ciliated sensory neurons

Curr Biol. 2001 Mar 20;11(6):457-61. doi: 10.1016/s0960-9822(01)00122-1.

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).

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Animals
  • Caenorhabditis elegans / genetics*
  • Caenorhabditis elegans / metabolism
  • Chlamydomonas
  • Flagella / metabolism
  • Gene Expression Profiling
  • Genes, Helminth / physiology*
  • Mice
  • Neurons, Afferent / metabolism
  • Plant Proteins
  • Polycystic Kidney, Autosomal Recessive / genetics*
  • Proteins / genetics*
  • Protozoan Proteins / genetics
  • Tumor Suppressor Proteins*

Substances

  • IFT88 protein, Chlamydomonas
  • Plant Proteins
  • Proteins
  • Protozoan Proteins
  • Tg737Rpw protein, mouse
  • Tumor Suppressor Proteins