PUBLICATION

Do cGMP Levels Drive the Speed of Photoreceptor Degeneration?

Authors
Iribarne, M., Masai, I.
ID
ZDB-PUB-180504-7
Date
2018
Source
Advances in experimental medicine and biology   1074: 327-333 (Chapter)
Registered Authors
Iribarne, Maria, Masai, Ichiro
Keywords
Mouse, Pde6, Photoreceptor degeneration, Rapid degeneration, Slow degeneration, Zebrafish, cGMP
MeSH Terms
  • Animals
  • Color Vision Defects/enzymology
  • Color Vision Defects/genetics
  • Cyclic GMP/physiology*
  • Cyclic Nucleotide Phosphodiesterases, Type 6/deficiency
  • Cyclic Nucleotide Phosphodiesterases, Type 6/genetics
  • Cyclic Nucleotide Phosphodiesterases, Type 6/physiology
  • Disease Models, Animal*
  • Eye Proteins
  • Forecasting
  • Humans
  • Light Signal Transduction
  • Photoreceptor Cells, Vertebrate/metabolism
  • Photoreceptor Cells, Vertebrate/pathology*
  • Retinal Degeneration/genetics
  • Retinal Degeneration/metabolism*
  • Zebrafish/genetics
  • Zebrafish/metabolism
  • Zebrafish Proteins/deficiency
  • Zebrafish Proteins/genetics
  • Zebrafish Proteins/physiology
PubMed
29721960 Full text @ Adv. Exp. Med. Biol.
Abstract
Humans with mutations in the phototransduction pathway develop forms of retinal degeneration, such as retinitis pigmentosa, cone dystrophy, or Leber congenital amaurosis. Similarly, numerous phototransduction mutant animal models resemble retinal degeneration. In our lab, using a zebrafish model, we study cone-specific phototransduction mutants. cGMP is the second messenger in the phototransduction pathway, and abnormal cGMP levels are associated with photoreceptor death. Rd1, a rod-specific phosphodiesterase 6 (Pde6) subunit mutant in mice, is one of the most widely used animal models for retinal degeneration. Rd1 mutant mice accumulate cGMP, causing rapid photoreceptor degeneration. However, much less is known about photoreceptor mutants producing abnormally low levels of cGMP. Here, focusing on Pde6 mutants in zebrafish and mice, we propose a correlation between cGMP levels and speed of photoreceptor degeneration.
Genes / Markers
Figures
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Antibodies
Orthology
Engineered Foreign Genes
Mapping