Characterisation of a type II functionally-deficient variant of alpha-1-antitrypsin discovered in the general population

Lung disease in alpha-1-antitrypsin deficiency (AATD) results from dysregulated proteolytic activity, mainly by neutrophil elastase (HNE), in the lung parenchyma. This is the result of a substantial reduction of circulating alpha-1-antitrypsin (AAT) and the presence in the plasma of inactive polymer...

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Sparad:
Bibliografiska uppgifter
I publikationen:PLoS One
Huvudupphovsmän: Laffranchi, Mattia, Elliston, Emma L. K., Gangemi, Fabrizio, Berardelli, Romina, Lomas, David A., Irving, James A., Fra, Annamaria
Materialtyp: Artigo
Språk:Inglês
Publicerad: Public Library of Science 2019
Ämnen:
Research Article
Länkar:https://ncbi.nlm.nih.gov/pmc/articles/PMC6329500/
https://ncbi.nlm.nih.gov/pubmed/30633749
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0206955
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