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Griciuc, A. ; Aron, L.* ; Ueffing, M.

ER stress in retinal degeneration: A target for rational therapy?

Trends Mol. Med. 17, 442-451 (2011)
DOI PMC
Open Access Green möglich sobald Postprint bei der ZB eingereicht worden ist.
Mutations that cause rhodopsin misfolding and retention within the endoplasmic reticulum (ER) are a prominent cause of retinitis pigmentosa. Here, we discuss the hypothesis that the failure of photoreceptor neurons to adapt to the stress caused by rhodopsin accumulation in the ER leads to a global collapse of homeostasis and to retinal degeneration. We review the molecular mechanisms underlying the activity of local ER conformational sensors and stress-relaying modules and consider how ER-derived stress signals are amplified and implemented to impact on downstream processes, including rhodopsin clearance and cell fate control. The emerging view is that alterations to the systems responsible for the detection, transduction and implementation of ER stress might be used therapeutically to treat retinitis pigmentosa.
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Publikationstyp Artikel: Journalartikel
Dokumenttyp Review
Korrespondenzautor
Schlagwörter endoplasmic-reticulum stress; unfolded protein response; dominant retinitis-pigmentosa; ubiquitin-proteasome system; cell-death; rhodopsin maturation; mutant rhodopsin; mouse model; in-vivo; degradation
ISSN (print) / ISBN 1471-4914
e-ISSN 1471-499X
Quellenangaben Band: 17, Heft: 8, Seiten: 442-451 Artikelnummer: , Supplement: ,
Verlag Elsevier
Verlagsort Oxford, UK
Nichtpatentliteratur Publikationen
Begutachtungsstatus Peer reviewed