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Mutations in the ubiquitin-binding domain of OPTN/optineurin interfere with autophagy-mediated degradation of misfolded proteins by a dominant-negative mechanism

OPTN (optineurin) is an autophagy receptor and mutations in the OPTN gene result in familial glaucoma (E50K) and amyotrophic lateral sclerosis (ALS) (E478G). However, the mechanisms through which mutant OPTN leads to human diseases remain to be characterized. Here, we demonstrated that OPTN colocali...

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Publicat a:Autophagy
Autors principals: Shen, Wen-Chuan, Li, Huei-Ying, Chen, Guang-Chao, Chern, Yijuang, Tu, Pang-hsien
Format: Artigo
Idioma:Inglês
Publicat: Taylor & Francis 2015
Matèries:
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC4502753/
https://ncbi.nlm.nih.gov/pubmed/25484089
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.4161/auto.36098
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