Retinitis Pigmentosa: Over-expression of anti-ageing protein Klotho in degenerating photoreceptors.

Forskningsoutput: TidskriftsbidragArtikel i vetenskaplig tidskrift

Abstract

Retinitis Pigmentosa involves a hereditary degeneration of photoreceptors by as yet unresolved mechanisms. The secretable protein α-Klotho has a function related to ageing processes, and α-Klotho deficient mice have reduced lifespan and declining functions in several tissues. Here, we studied Klotho in connection with inherited photoreceptor degeneration. Increased nuclear immunostaining for α-Klotho protein was seen in degenerating photoreceptors in four different Retinitis Pigmentosa models (rd1, rd2 mice; P23H, S334ter rhodopsin mutant rats). Correspondingly, in rd1 retina α-Klotho mRNA expression was significantly upregulated. Moreover, immunostaining for another Klotho family protein, β-Klotho, also co-localised with degenerating rd1 photoreceptors. The rd1 retina displayed reduced levels of fibroblast growth factor (FGF) 15, a member of the FGF subfamily for which Klotho acts as a co-receptor. Exogenous α-Klotho protein added to retinal explant cultures did not affect cell death in rd1 retinae, but caused a severe layer disordering in wild-type retinae. Our work suggests Klotho as a novel player in the retina, with a clear connection to photoreceptor cell death as well as with an influence on retinal organization. This article is protected by copyright. All rights reserved.

Detaljer

Författare
  • Pietro Farinelli
  • Blanca Arango-Gonzalez
  • Jakob Völkl
  • Ioana Alesutan
  • Florian Lang
  • Eberhart Zrenner
  • Francois Paquet-Durand
  • Per Ekström
Enheter & grupper
Forskningsområden

Ämnesklassifikation (UKÄ) – OBLIGATORISK

  • Neurovetenskaper
Originalspråkengelska
TidskriftJournal of Neurochemistry
Tidigt onlinedatum2013 jun 24
StatusPublished - 2013
PublikationskategoriForskning
Peer review utfördJa