Matrix metalloproteinase activity in the lung is increased in Hermansky-Pudlak syndrome

BACKGROUND: Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disorder characterized by oculocutaneous albinism and platelet dysfunction and can sometimes lead to a highly aggressive form of pulmonary fibrosis that mimics the fatal lung condition called idiopathic pulmonary fibrosis (IPF...

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Detalles Bibliográficos
Autores principales: Summer, Ross, Krishna, Rachana, Schriner, DeLeila, Cuevas-Mora, Karina, Sales, Dominic, Para, Rachel, Roman, Jesse, Nieweld, Carl, Gochuico, Bernadette R., Romero, Freddy
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
Materias:
Research
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6610946/
https://www.ncbi.nlm.nih.gov/pubmed/31272455
http://dx.doi.org/10.1186/s13023-019-1143-0

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