Restoration of visual function in P23H rhodopsin transgenic rats by gene delivery of BiP/Grp78

The P23H mutation within the rhodopsin gene (RHO) causes rhodopsin misfolding, endoplasmic reticulum (ER) stress, and activates the unfolded protein response (UPR), leading to rod photoreceptor degeneration and autosomal dominant retinitis pigmentosa (ADRP). Grp78/BiP is an ER-localized chaperone th...

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Autors principals: Gorbatyuk, Marina. S., Knox, Tessa, LaVail, Matthew M., Gorbatyuk, Oleg S., Noorwez, Syed M., Hauswirth, William W., Lin, Jonathan H., Muzyczka, Nicholas, Lewin, Alfred S.
Format: Artigo
Idioma:Inglês
Publicat: National Academy of Sciences 2010
Matèries:
Biological Sciences
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC2851865/
https://ncbi.nlm.nih.gov/pubmed/20231467
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.0911991107
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