RGD Reference Report - Retinitis Pigmentosa: over-expression of anti-ageing protein Klotho in degenerating photoreceptors. - Rat Genome Database

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

Authors: Farinelli, P  Arango-Gonzalez, B  Volkl, J  Alesutan, I  Lang, F  Zrenner, E  Paquet-Durand, F  Ekstrom, PA 
Citation: Farinelli P, etal., J Neurochem. 2013 Dec;127(6):868-79. doi: 10.1111/jnc.12353. Epub 2013 Jul 22.
RGD ID: 10403060
Pubmed: PMID:23796581   (View Abstract at PubMed)
DOI: DOI:10.1111/jnc.12353   (Journal Full-text)

Retinitis Pigmentosa involves a hereditary degeneration of photoreceptors by as yet unresolved mechanisms. The secretable protein alpha-Klotho has a function related to ageing processes, and alpha-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 alpha-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 alpha-Klotho mRNA expression was significantly up-regulated. Moreover, immunostaining for another Klotho family protein, beta-Klotho, also co-localized with degenerating rd1 photoreceptors. The rd1 retina displayed reduced levels of fibroblast growth factor 15, a member of the fibroblast growth factor subfamily for which Klotho acts as a co-receptor. Exogenous alpha-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 study 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.

RGD Manual Disease Annotations    Click to see Annotation Detail View
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
retinitis pigmentosa  ISOKl (Rattus norvegicus)10403060; 10403060protein:increased expression:retina more ...RGD 
retinitis pigmentosa  ISOKl (Mus musculus)10403060; 10403060protein:increased expression:retina more ...RGD 
retinitis pigmentosa  ISOKlb (Mus musculus)10403060; 10403060protein:increased expression:outer nuclear layer:RGD 
retinitis pigmentosa  IEP 10403060; 10403060protein:increased expression:retina more ...RGD 
retinitis pigmentosa  IEP 10403060protein:increased expression:outer nuclear layer:RGD 

Objects Annotated

Genes (Rattus norvegicus)
Kl  (Klotho)
Klb  (klotho beta)

Genes (Mus musculus)
Kl  (klotho)
Klb  (klotho beta)

Genes (Homo sapiens)
KL  (klotho)
KLB  (klotho beta)


Additional Information