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Inactivation of VCP/ter94 Suppresses Retinal Pathology Caused by Misfolded Rhodopsin in Drosophila

The most common Rhodopsin (Rh) mutation associated with autosomal dominant retinitis pigmentosa (ADRP) in North America is the substitution of proline 23 by histidine (Rh(P23H)). Unlike the wild-type Rh, mutant Rh(P23H) exhibits folding defects and forms intracellular aggregates. The mechanisms resp...

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Detalles Bibliográficos
Autores principales: Griciuc, Ana, Aron, Liviu, Roux, Michel J., Klein, Rüdiger, Giangrande, Angela, Ueffing, Marius
Formato: Texto
Lenguaje:English
Publicado: Public Library of Science 2010
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2928793/
https://www.ncbi.nlm.nih.gov/pubmed/20865169
http://dx.doi.org/10.1371/journal.pgen.1001075