Abstract：
:RHO (Rod opsin) encodes a G-protein coupled receptor that is expressed exclusively by rod photoreceptors of the retina and forms the essential photopigment, rhodopsin, when coupled with 11-cis-retinal. Many rod opsin disease -mutations cause rod opsin protein misfolding and trigger endoplasmic reticulum (ER) stress, leading to activation of the Unfolded Protein Response (UPR) signal transduction network. Chop is a transcriptional activator that is induced by ER stress and promotes cell death in response to chronic ER stress. Here, we examined the role of Chop in transgenic mice expressing human P23H rhodopsin (hP23H Rho Tg) that undergo retinal degeneration. With the exception of one time point, we found no significant induction of Chop in these animals and no significant change in retinal degeneration by histology and electrophysiology when hP23H Rho Tg animals were bred into a Chop (-/-) background. Our results indicate that Chop does not play a significant causal role during retinal degeneration in these animals. We suggest that other modules of the ER stress-induced UPR signaling network may be involved photoreceptor disease induced by P23H rhodopsin.
journal_name
Adv Exp Med Bioljournal_title
Advances in experimental medicine and biologyauthors
Chiang WC,Joseph V,Yasumura D,Matthes MT,Lewin AS,Gorbatyuk MS,Ahern K,LaVail MM,Lin JHdoi
10.1007/978-3-319-17121-0_25subject
Has Abstractpub_date
2016-01-01 00:00:00pages
185-91eissn
0065-2598issn
2214-8019journal_volume
854pub_type
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