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Exosome Secretion Ameliorates Lysosomal Storage of Cholesterol in Niemann-Pick Type C Disease

Niemann-Pick type C1 disease is an autosomal-recessive lysosomal storage disorder. Loss of function of the npc1 gene leads to abnormal accumulation of free cholesterol and sphingolipids within the late endosomal and lysosomal compartments resulting in progressive neurodegeneration and dysmyelination...

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Detalhes bibliográficos
Main Authors: Strauss, Katrin, Goebel, Cornelia, Runz, Heiko, Möbius, Wiebke, Weiss, Sievert, Feussner, Ivo, Simons, Mikael, Schneider, Anja
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Biochemistry and Molecular Biology 2010
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2924046/
https://ncbi.nlm.nih.gov/pubmed/20554533
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M110.134775
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