Mouse models to study GCAP functions in intact photoreceptors

Adv Exp Med Biol. 2002:514:361-88. doi: 10.1007/978-1-4615-0121-3_22.

Abstract

In photoreceptor cells cGMP is the second messenger that transduces light into an electrical response. Regulation of cGMP synthesis by Ca2+ is one of the key mechanisms by which Ca2+ exerts negative feedback to the phototransduction cascade in the process of light adaptation. This Ca2+ feedback to retinal guanylyl cyclases (Ret-GCs) is conferred by the guanylate cyclase-activating proteins (GCAPs). Mutations in GCAP1 that disrupt the Ca2+ regulation of Ret-GCs in vitro have been associated with severe human vision disorders. This chapter focuses on recent data obtained from biochemical and electrophysiological studies of GCAP1/GCAP2 knockout mice and other GCAP transgenic mice, addressing: 1. the quantitative aspects of the Ca2+-feedback to Ret-GCs in regulating the light sensitivity and adaptation in intact rods; 2. functional differences between GCAP1 and GCAP2 in intact rod photoreceptors; and 3. whether GCAP mutants with impaired Ca2+ binding lead to retinal disease in vivo by constitutive activation of Ret-GCs and elevation of intracellular cGMP, as predicted from in vitro studies.

Publication types

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

MeSH terms

  • Animals
  • Calcium / metabolism
  • Calcium-Binding Proteins / genetics*
  • Calcium-Binding Proteins / metabolism
  • Calcium-Binding Proteins / physiology*
  • Disease Models, Animal
  • Electrophysiology
  • Guanylate Cyclase-Activating Proteins
  • Humans
  • Light
  • Mice
  • Mice, Knockout
  • Mice, Transgenic
  • Models, Biological
  • Mutation
  • Phenotype
  • Photons
  • Retina / metabolism
  • Retina / physiology*
  • Retinal Diseases / genetics
  • Time Factors

Substances

  • Calcium-Binding Proteins
  • GUCA1A protein, human
  • GUCA1B protein, human
  • Guanylate Cyclase-Activating Proteins
  • Guca1a protein, mouse
  • Guca1b protein, mouse
  • Calcium