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

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

Abstract 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 fibro...

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Bibliographic Details
Main Authors: Ross Summer, Rachana Krishna, DeLeila Schriner, Karina Cuevas-Mora, Dominic Sales, Rachel Para, Jesse Roman, Carl Nieweld, Bernadette R. Gochuico, Freddy Romero
Format: Article
Language:English
Published: BMC 2019-07-01
Series:Orphanet Journal of Rare Diseases
Subjects:
Hermansky-Pudlak syndrome
Matrix metalloproteinase
Pulmonary fibrosis
Online Access:http://link.springer.com/article/10.1186/s13023-019-1143-0

Internet

http://link.springer.com/article/10.1186/s13023-019-1143-0

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