Article
Function of a rare variant of alpha-1-antitrypsin, phenotype P(i) EFranklin S, a poor inhibitor of human neutrophil elastase.
Research in experimental medicine. Zeitschrift fur die gesamte experimentelle Medizin einschliesslich experimenteller Chirurgie - 1 Jan 1997
Cook L, Knight K R, Burdon J G, Brenton S, Hunt J M
Abstract excerpt
A 31-year-old woman with the rare alpha-1-antitrypsin (A1AT) phenotype P(i) EFranklin S presented to this laboratory. Since little is known about the EFranklin protein, a study was established to investigate the biochemical properties of this glycoprotein, notably its inhibitory activity against human neutrophil elastase (HNE), compared with that of the more common A1AT variants M and Z. The serum A1AT level of...
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