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Histone deacetylase inhibitors prevent the degradation and restore the activity of glucocerebrosidase in Gaucher disease

Gaucher disease (GD) is caused by a spectrum of genetic mutations within the gene encoding the lysosomal enzyme glucocerebrosidase (GCase). These mutations often lead to misfolded proteins that are recognized by the unfolded protein response system and are degraded through the ubiquitin–proteasome p...

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Autori principali: Lu, Jie, Yang, Chunzhang, Chen, Masako, Ye, Donald Y., Lonser, Russell R., Brady, Roscoe O., Zhuang, Zhengping
Natura: Artigo
Lingua:Inglês
Pubblicazione: National Academy of Sciences 2011
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC3248545/
https://ncbi.nlm.nih.gov/pubmed/22160715
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.1119181109
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