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NPC1 late endosomes contain elevated levels of non-esterified (‘free’) fatty acids and an abnormally glycosylated form of the NPC2 protein

NPC (Niemann–Pick type C) disease is a rare lipidosis characterized by the accumulation of LDL (low-density lipoprotein)-derived non-esterified cholesterol in the E/L (endosomal/lysosomal) system. The gene products that are responsible for the two NPC complementation groups are distinct and dissimil...

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Detalhes bibliográficos
Main Authors: Chen, Fannie W., Gordon, Ronald E., Ioannou, Yiannis A.
Formato: Artigo
Idioma:Inglês
Publicado em: Portland Press Ltd. 2005
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC1198935/
https://ncbi.nlm.nih.gov/pubmed/15896196
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1042/BJ20050236
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