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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 as soon as Postprint is submitted to ZB.
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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Publication type Article: Journal article
Document type Review
Keywords endoplasmic-reticulum stress; unfolded protein response; dominant retinitis-pigmentosa; ubiquitin-proteasome system; cell-death; rhodopsin maturation; mutant rhodopsin; mouse model; in-vivo; degradation
Language english
Publication Year 2011
HGF-reported in Year 2011
ISSN (print) / ISBN 1471-4914
e-ISSN 1471-499X
Journal Trends in Molecular Medicine
Quellenangaben Volume: 17, Issue: 8, Pages: 442-451 Article Number: , Supplement: ,
Publisher Elsevier
Publishing Place Oxford, UK
Reviewing status Peer reviewed
Institute(s) CF Metabolomics & Proteomics (CF-MPC)
POF-Topic(s) 30203 - Molecular Targets and Therapies
Research field(s) Enabling and Novel Technologies
PSP Element(s) G-505700-001
PubMed ID 21620769
DOI 10.1016/j.molmed.2011.04.002
Scopus ID 80955178939
Erfassungsdatum 2011-11-07
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