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Article

Alpha-1 Antitrypsin-Deficient Macrophages Have Increased Matriptase-Mediated Proteolytic Activity.

American journal of respiratory cell and molecular biology - 1 Aug 2017

Krotova Karina, Marek George W, Wang Rejean L, Aslanidi George, Hoffman Brad E, Khodayari Nazli, Rouhani Farshid N, Brantly Mark L

Abstract excerpt

Alpha-1 antitrypsin (AAT) deficiency-associated emphysema is largely attributed to insufficient inhibition of neutrophil elastase released from neutrophils. Correcting AAT levels using augmentation therapy only slows disease progression, and that suggests a more complex process of lung destruction. Because alveolar macrophages (Mɸ) express AAT, we propose that the expression and intracellular accumulation of...

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Identifiers

PMID: 28362108
DOI: 10.1165/rcmb.2016-0366OC
PMCID: PMC5800887

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