Elastase regulates the synthesis of its inhibitor, alpha 1-proteinase inhibitor, and exaggerates the defect in homozygous PiZZ alpha 1 PI deficiency.

The net balance of neutrophil elastase, an enzyme that degrades many components of the extracellular matrix, and its inhibitor, alpha-1-proteinase inhibitor (alpha 1 PI), is thought to be a critical determinant in the development of destructive lung disease, especially in individuals with homozygous...

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Veröffentlicht in:	J Clin Invest
Hauptverfasser:	Perlmutter, D H, Travis, J, Punsal, P I
Format:	Artigo
Sprache:	Inglês
Veröffentlicht:	American Society for Clinical Investigation 1988
Schlagworte:	Research Article
Online-Zugang:	https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC442624/ https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/3260245/ https://ncbi.nlm.nih.govhttps://doi.org/10.1172/JCI113519
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