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FKBP10 Depletion Enhances Glucocerebrosidase Proteostasis in Gaucher's Disease Fibroblasts
Lysosomal storage diseases (LSDs) are often caused by mutations compromising lysosomal enzyme folding in the endoplasmic reticulum (ER), leading to degradation and loss of function. Mass spectrometry analysis of Gaucher fibroblasts treated with mechanistically distinct molecules that increase LSD en...
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| Autores principales: | , , , , , |
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| Formato: | Artigo |
| Lenguaje: | Inglês |
| Publicado: |
2013
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| Materias: | |
| Acceso en línea: | https://ncbi.nlm.nih.gov/pmc/articles/PMC3624024/ https://ncbi.nlm.nih.gov/pubmed/23434032 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.chembiol.2012.11.014 |
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