46 Scopus citations


α1-Antitrypsin (α1-AT) is the major endogenous inhibitor of neutrophil elastase. Individuals with α1-AT deficiency are susceptible to premature development of emphysema. Thus a greater understanding of this serine proteinase inhibitor (serpin) has been a major objective of research on the pathogenesis of emphysema. In this article, we review recent literature on the α1-AT gene and its relationship to other members of the serpin supergene family, particularly as it pertains to the function of α1-AT. We also discuss the current literature on biosynthesis of α1-AT and how its synthesis may be tightly regulated by the net balance of neutrophil elastase and α1-AT at sites of inflammation/tissue injury. The net functional activity of α1-AT in complex biological fluids is also affected by interaction with other enzymes, inhibitors, matrix proteins, and endogenous oxidants. Finally, we discuss the pathogenesis, clinical manifestations, and treatment of injury to the lung associated with deficiency variants of the α1-AT gene.

Original languageEnglish
Pages (from-to)1/2
JournalAmerican Journal of Physiology - Lung Cellular and Molecular Physiology
Issue number3
StatePublished - 1989


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