Abstract
Twenty-six different alleles have been identified for alpha 1 protease inhibitor (alpha 1 antitrypsin), each designated by a letter of the alphabet. In any individual two alleles codominantly determine the characteristics of alpha 1 protease inhibitor (Pi), including mobility on electrophoresis, serum concentration and acute phase response. Recent evidence has linked some mildly deficient phenotypes of Pi with a variety of chronic immunologic and inflammatory disorders, such as rheumatoid arthritis, juvenile chronic arthritis, systemic lupus erythematosus, ankylosing spondylitis, uveitis, asthma and fibrosing alveolitis, in addition to the well recognised association of severe deficiency with emphysema and chronic liver disease. This disease susceptibility in phenotypes associated with reduced serum levels may be due to alteration in lymphocyte responses, complement activation and leukocyte migration. Pi can also influence the autolytic effects of leukocytic enzymes on tissues and may inhibit some aspects of coagulation and fibrinolysis. Therefore patients with deficient Pi phenotypes are likely to have exaggerated immunologic and inflammatory responses.
References
Nov 19, 1977·Lancet·D M GeddesA M Ward
Dec 24, 1977·Lancet·P D BuisseretM H Lessof
May 20, 1978·Lancet·D A BrewertonA M Ward
Feb 14, 1978·Cell and Tissue Research·M B Ray, V J Desmet
Jun 1, 1977·Annals of Internal Medicine·H M RubinsteinF R Schmid
Aug 10, 1978·Nature·P K AroraL D Aronson
Dec 1, 1977·The Journal of Clinical Investigation·P ArnaudW P Faulk
Jan 1, 1978·Life Sciences·L Benítez-Bribiesca, R Freyre-Horta
Jan 1, 1976·Transplantation Reviews·M TakahashiV Nussenzweig
Jan 1, 1975·Advances in Experimental Medicine and Biology·A D BorsodiR M Bruce
Jan 17, 1976·British Medical Journal
Jun 1, 1976·FEBS Letters·J O Jeppsson
Jul 1, 1976·Annals of Internal Medicine·H J FreemanF A Herbert
Dec 21, 1972·The New England Journal of Medicine·N O Berg, S Eriksson
Nov 1, 1973·The Journal of Clinical Investigation·A B Cohen
Dec 1, 1974·The Journal of Experimental Medicine·L W Heck, A P Kaplan
Feb 1, 1973·The Journal of Clinical Investigation·P A Ward, R C Talamo
Jan 1, 1968·Humangenetik·F Kueppers
Aug 27, 1965·Science·M K Fagerhol, M Braend
Citations
Oct 1, 1985·The American Journal of Medicine·M LewisA Meyers
Jan 1, 1985·International Journal of Immunopharmacology·R Müller-PeddinghausW Hillebrand
Jan 1, 1986·Comparative Biochemistry and Physiology. B, Comparative Biochemistry·A DubinA Koj
Jan 1, 1984·The Journal of Experimental Medicine·Z LiaoD L Rosenstreich
Apr 17, 2001·American Journal of Therapeutics·J PrandotaL Kotecki
May 1, 1995·Acta Neurologica Scandinavica·Y I Lolin, A M Ward
Feb 1, 1992·Journal of Clinical Periodontology·E AdonogianakiD F Kinane
Jul 1, 1985·The British Journal of Ophthalmology·D WakefieldR Penny
Jan 1, 1983·British Heart Journal·H GilutzM R Quastel
Nov 1, 1982·Journal of Clinical Pathology·F CalleaV J Desmet
Oct 1, 1982·Chest·D W Cockcroft, S L Horne
Jan 1, 1986·Journal of Clinical Periodontology·L Sandholm
Aug 1, 1983·Journal of Immunogenetics·M R Laurent, K I Welsh
Jun 1, 1985·Clinical Immunology and Immunopathology·S N BreitR Penny
Apr 12, 2005·Clinical Biochemistry·Saeid GhavamiEnrique Fernandez Bustillo
Sep 29, 2017·Current Diabetes Reports·James C Needell, Danny Zipris
Sep 11, 2004·International Journal of Dermatology·Priscila de OliveiraNelson H Tatsui
Sep 1, 1983·American Journal of Physical Anthropology·B K Suarez, J A Pierce
May 1, 1994·Journal of Periodontal Research·E AdonogianakiD F Kinane
Feb 1, 1996·Journal of Periodontal Research·E AdonogianakiD F Kinane
May 1, 1991·Journal of Periodontal Research·D F KinaneM Thornhill
Jan 1, 1989·Critical Reviews in Clinical Laboratory Sciences·R D Feld
Dec 1, 2012·Diabetes, Obesity & Metabolism·D A SchneiderM G von Herrath
Dec 1, 1982·Arthritis and Rheumatism·D WakefieldR Penny
Mar 6, 1982·The Medical Journal of Australia·D WakefieldR Penny
Mar 4, 1982·Biochimica Et Biophysica Acta·L VaughanR W Carrell