Photoreceptor outer segment as a sink for membrane proteins: hypothesis and implications in retinal ciliopathies

Hum Mol Genet. 2017 Aug 1;26(R1):R75-R82. doi: 10.1093/hmg/ddx163.

Abstract

The photoreceptor outer segment (OS) is a unique modification of the primary cilium, specialized for light perception. Being homologous organelles, the primary cilium and the OS share common building blocks and molecular machinery to construct and maintain them. The OS, however, has several unique structural features that are not seen in primary cilia. Although these unique features of the OS have been well documented, their implications in protein localization have been under-appreciated. In this review, we compare the structural properties of the primary cilium and the OS, and propose a hypothesis that the OS can act as a sink for membrane proteins. We further discuss the implications of this hypothesis in polarized protein localization in photoreceptors and mechanisms of photoreceptor degeneration in retinal ciliopathies.

Publication types

  • Review
  • Research Support, N.I.H., Extramural

MeSH terms

  • Animals
  • Cilia / metabolism
  • Cilia / physiology
  • Ciliopathies / metabolism*
  • Humans
  • Membrane Proteins / metabolism
  • Photoreceptor Cells / metabolism
  • Photoreceptor Cells, Vertebrate / metabolism
  • Photoreceptor Cells, Vertebrate / pathology
  • Protein Transport
  • Retina / metabolism
  • Retina / physiology*
  • Retinal Degeneration / metabolism

Substances

  • Membrane Proteins