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Loss of Niemann-Pick C1 or C2 Protein Results in Similar Biochemical Changes Suggesting That These Proteins Function in a Common Lysosomal Pathway

Niemann-Pick Type C (NPC) disease is a lysosomal storage disorder characterized by accumulation of unesterified cholesterol and other lipids in the endolysosomal system. NPC disease results from a defect in either of two distinct cholesterol-binding proteins: a transmembrane protein, NPC1, and a sma...

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Detalles Bibliográficos
Autores principales: Dixit, Sayali S., Jadot, Michel, Sohar, Istvan, Sleat, David E., Stock, Ann M., Lobel, Peter
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3161064/
https://www.ncbi.nlm.nih.gov/pubmed/21887293
http://dx.doi.org/10.1371/journal.pone.0023677