Membrane-binding and enzymatic properties of RPE65

Prog Retin Eye Res. 2010 Sep;29(5):428-42. doi: 10.1016/j.preteyeres.2010.03.002. Epub 2010 Mar 19.

Abstract

Regeneration of visual pigments is essential for sustained visual function. Although the requirement for non-photochemical regeneration of the visual chromophore, 11-cis-retinal, was recognized early on, it was only recently that the trans to cis retinoid isomerase activity required for this process was assigned to a specific protein, a microsomal membrane enzyme called RPE65. In this review, we outline progress that has been made in the functional characterization of RPE65. We then discuss general concepts related to protein-membrane interactions and the mechanism of the retinoid isomerization reaction and describe some of the important biochemical and structural features of RPE65 with respect to its membrane-binding and enzymatic properties.

Publication types

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

MeSH terms

  • Animals
  • Carrier Proteins / chemistry*
  • Carrier Proteins / metabolism
  • Eye Proteins / chemistry*
  • Eye Proteins / metabolism
  • Humans
  • Leber Congenital Amaurosis / metabolism
  • Protein Binding / physiology
  • Retinal Pigment Epithelium / enzymology
  • cis-trans-Isomerases

Substances

  • Carrier Proteins
  • Eye Proteins
  • retinoid isomerohydrolase
  • cis-trans-Isomerases