Endosomal/Lysosomal Processing of Gangliosides Affects Neuronal Cholesterol Sequestration in Niemann-Pick Disease Type C

Niemann-Pick disease type C (NPC) is a severe neurovisceral lysosomal storage disorder caused by defects in NPC1 or NPC2 proteins. Although numerous studies support the primacy of cholesterol storage, neurons of double-mutant mice lacking both NPC1 and an enzyme required for synthesis of all complex...

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Главные авторы: Zhou, Sharon, Davidson, Cristin, McGlynn, Robert, Stephney, Gloria, Dobrenis, Kostantin, Vanier, Marie T., Walkley, Steven U.
Формат: Artigo
Язык:Inglês
Опубликовано: American Society for Investigative Pathology 2011
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Regular Article
Online-ссылка:https://ncbi.nlm.nih.gov/pmc/articles/PMC3157170/
https://ncbi.nlm.nih.gov/pubmed/21708114
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.ajpath.2011.04.017
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