Impaired Lysosomal Integral Membrane Protein 2-dependent Peroxiredoxin 6 Delivery to Lamellar Bodies Accounts for Altered Alveolar Phospholipid Content in Adaptor Protein-3-deficient pearl Mice

The Hermansky Pudlak syndromes (HPS) constitute a family of disorders characterized by oculocutaneous albinism and bleeding diathesis, often associated with lethal lung fibrosis. HPS results from mutations in genes of membrane trafficking complexes that facilitate delivery of cargo to lysosome-relat...

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Detalhes bibliográficos
Publicado no:J Biol Chem
Main Authors: Kook, Seunghyi, Wang, Ping, Young, Lisa R., Schwake, Michael, Saftig, Paul, Weng, Xialian, Meng, Ying, Neculai, Dante, Marks, Michael S., Gonzales, Linda, Beers, Michael F., Guttentag, Susan
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Biochemistry and Molecular Biology 2016
Assuntos:
Cell Biology
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC4861416/
https://ncbi.nlm.nih.gov/pubmed/26907692
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M116.720201
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