Structural evidence for methionine at the reactive site of human alpha-1-proteinase inhibitor.

Alpha 1-proteinase inhibitor (human) shortage.

Long-term clinical outcomes following treatment with alpha 1-proteinase inhibitor for COPD associated with alpha-1 antitrypsin deficiency: a look at the evidence

Alpha-1 antitrypsin deficiency (AATD) is a common hereditary disorder caused by mutations in the SERPINA1 gene, which encodes alpha-1 antitrypsin (AAT; also known as alpha 1-proteinase inhibitor, A1-PI). An important function of A1-PI in the lung is to inhibit neutrophil elastase, one of various proteolytic enzymes released by activated neutrophils during inflammation. Absence or deficiency of ...

Lipopolysaccharide modulates the expression of alpha 1 proteinase inhibitor and other serine proteinase inhibitors in human monocytes and macrophages

alpha 1 Proteinase inhibitor (PI) is the principle inhibitor of neutrophil elastase, an enzyme that degrades many components of the extracellular matrix. Expression and regulation of alpha 1 PI, therefore, affects the delicate balance of elastase and antielastase, which is critical to turnover of connective tissue during homeostasis, tissue injury, and repair. In this study we show that express...

Primary structure of the reactive site of human C1-inhibitor.

Human C1-inhibitor (C1-Inh) forms an equimolar complex with complement proteinase C1s that is resistant to dissociation by sodium dodecyl sulfate. The formation of this stable complex results in the cleavage of a peptide bond near the carboxyl terminus of the inhibitor and, whereas the bulk of C1-Inh remains covalently bound to the light chain of C1s, the postcomplex inhibitor peptide can be is...

The Oxidative Inactivation of Human a-1-Proteinase Inhibitor

We have previously shown that the reactive center of human a-1-proteinase inhibitor (a-l-PI) contains a methionine at position P1 (Johnson, D., and Travis, J. (1978) J. Bid. Chem 263,7142-7144). The importance of this residue has been tested by oxidizing the inhibitor with increasing concentrations of N-chlorosuccinimide (SucNCl). Under the conditions utilized a maximum of 2 of the 8 methionyl ...