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Pharmacological Inhibition of the VCP/Proteasome Axis Rescues Photoreceptor Degeneration in RHO(P23H) Rat Retinal Explants
Rhodopsin (RHO) misfolding mutations are a common cause of the blinding disease autosomal dominant retinitis pigmentosa (adRP). The most prevalent mutation, RHO(P23H), results in its misfolding and retention in the endoplasmic reticulum (ER). Under homeostatic conditions, misfolded proteins are sele...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8534135/ https://www.ncbi.nlm.nih.gov/pubmed/34680161 http://dx.doi.org/10.3390/biom11101528 |