Routes and machinery of primary cilium biogenesis

Cell Mol Life Sci. 2017 Nov;74(22):4077-4095. doi: 10.1007/s00018-017-2570-5. Epub 2017 Jun 17.

Abstract

Primary cilia are solitary, microtubule-based protrusions of the cell surface that play fundamental roles as photosensors, mechanosensors and biochemical sensors. Primary cilia dysfunction results in a long list of developmental and degenerative disorders that combine to give rise to a large spectrum of human diseases affecting almost any major body organ. Depending on the cell type, primary ciliogenesis is initiated intracellularly, as in fibroblasts, or at the cell surface, as in renal polarized epithelial cells. In this review, we have focused on the routes of primary ciliogenesis placing particular emphasis on the recently described pathway in renal polarized epithelial cells by which the midbody remnant resulting from a previous cell division event enables the centrosome for initiation of primary cilium assembly. The protein machinery implicated in primary cilium formation in epithelial cells, including the machinery best known for its involvement in establishing cell polarity and polarized membrane trafficking, is also discussed.

Keywords: Alternative pathway; Fibroblasts; Intracellular pathway; Midbody remnant; Polarized epithelial cells; Primary ciliogenesis; Protein machinery.

Publication types

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

MeSH terms

  • Animals
  • Bardet-Biedl Syndrome / metabolism
  • Bardet-Biedl Syndrome / pathology
  • Cell Membrane / metabolism
  • Cell Polarity
  • Cilia / chemistry
  • Cilia / metabolism*
  • Humans
  • Mechanotransduction, Cellular
  • Membrane Proteins / metabolism
  • Microtubule-Associated Proteins / metabolism
  • Tight Junctions / metabolism
  • rab GTP-Binding Proteins / metabolism

Substances

  • Membrane Proteins
  • Microtubule-Associated Proteins
  • rab GTP-Binding Proteins