Open Access Green möglich sobald Postprint bei der ZB eingereicht worden ist.
ER stress in retinal degeneration: A target for rational therapy?
Trends Mol. Med. 17, 442-451 (2011)
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.
Altmetric
Weitere Metriken?
Zusatzinfos bearbeiten
[➜Einloggen]
Publikationstyp
Artikel: Journalartikel
Dokumenttyp
Review
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
Zeitschrift
Trends in Molecular Medicine
Quellenangaben
Band: 17,
Heft: 8,
Seiten: 442-451
Verlag
Elsevier
Verlagsort
Oxford, UK
Nichtpatentliteratur
Publikationen
Begutachtungsstatus
Peer reviewed
Institut(e)
CF Metabolomics & Proteomics (CF-MPC)