α1-Antitrypsin: more than just deficiency
- Correspondence to:
Professor R A Stockley
Department of Medicine, Queen Elizabeth Hospital, Edgbaston, Birmingham B15 2TH, UK; r.a.stockleybham.ac.uk
Abnormal levels of α1-antitrypsin represent a syndrome of clinical disease entities, some relating to a deficiency while others reflect an overload
Carl-Bertil Laurell (1919–2001) was head of the Clinical Chemistry Department at Malmö General Hospital, University of Lund, Sweden (1954–84) and continued working in the department until his death in 2001. He had an interest in the initial studies of protein biochemistry, and his early paper on electrophoresis studies of serum proteins led to the discovery of subjects with deficient bands in the α1-globulin region.1 This region showed the greatest inhibition of trypsin, and the major protein within the band became known as α1-antitrypsin. Having identified several subjects with a weak α1 band seen on paper electrophoresis, Laurell and his research fellow Eriksson investigated the patients further. Three of the original five patients had severe early onset pulmonary emphysema suggesting a cause and effect.1 For many years research focused on understanding the role of this protein in the pathogenesis of emphysema. …
