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Unfolded protein response in Gaucher disease: from human to Drosophila

BACKGROUND: In Gaucher disease (GD), resulting from mutations in the GBA gene, mutant β-glucocerebrosidase (GCase) molecules are recognized as misfolded in the endoplasmic reticulum (ER). They are retrotranslocated to the cytoplasm, where they are ubiquitinated and undergo proteasomal degradation in...

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Autores principales: Maor, Gali, Rencus-Lazar, Sigal, Filocamo, Mirella, Steller, Hermann, Segal, Daniel, Horowitz, Mia
Formato: Artigo
Lenguaje:Inglês
Publicado: BioMed Central 2013
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Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC3819655/
https://ncbi.nlm.nih.gov/pubmed/24020503
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/1750-1172-8-140
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