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

Farinelli, Pietro LU ; Arango-Gonzalez, Blanca; Völkl, Jakob; Alesutan, Ioana; Lang, Florian; Zrenner, Eberhart; Paquet-Durand, Francois LU and Ekström, Per LU (2013) In Journal of Neurochemistry
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... (More)
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. (Less)
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Contribution to journal
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published
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Journal of Neurochemistry
publisher
Wiley-Blackwell
external identifiers
  • PMID:23796581
  • Scopus:84890127736
ISSN
1471-4159
DOI
10.1111/jnc.12353
language
English
LU publication?
yes
id
e57f68c4-da64-4963-bba4-44224ea1c3b8 (old id 3913032)
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http://www.ncbi.nlm.nih.gov/pubmed/23796581?dopt=Abstract
date added to LUP
2013-07-01 15:22:59
date last changed
2016-10-13 04:24:49
@misc{e57f68c4-da64-4963-bba4-44224ea1c3b8,
  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.},
  author       = {Farinelli, Pietro and Arango-Gonzalez, Blanca and Völkl, Jakob and Alesutan, Ioana and Lang, Florian and Zrenner, Eberhart and Paquet-Durand, Francois and Ekström, Per},
  issn         = {1471-4159},
  language     = {eng},
  month        = {06},
  publisher    = {ARRAY(0x8bfef20)},
  series       = {Journal of Neurochemistry},
  title        = {Retinitis Pigmentosa: Over-expression of anti-ageing protein Klotho in degenerating photoreceptors.},
  url          = {http://dx.doi.org/10.1111/jnc.12353},
  year         = {2013},
}