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Hermansky-Pudlak Syndrome and Lung Disease: Pathogenesis and Therapeutics

Hermansky-Pudlak Syndrome (HPS) is a rare, genetic, multisystem disorder characterized by oculocutaneous albinism (OCA), bleeding diathesis, immunodeficiency, granulomatous colitis, and pulmonary fibrosis. HPS pulmonary fibrosis (HPS-PF) occurs in 100% of patients with subtype HPS-1 and has a simila...

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Detalles Bibliográficos
Autores principales:	Velázquez-Díaz, Pamela, Nakajima, Erika, Sorkhdini, Parand, Hernandez-Gutierrez, Ashley, Eberle, Adam, Yang, Dongqin, Zhou, Yang
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Frontiers Media S.A. 2021
Materias:	Pharmacology
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8028140/ https://www.ncbi.nlm.nih.gov/pubmed/33841163 http://dx.doi.org/10.3389/fphar.2021.644671

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