Alpha-1 Antitrypsin (Alpha-1-Proteinase Inhibitor/AAT/API)

Alpha 1 Antitrypsin Human Plasma Partially Purified
  • 5 mg
  • 100 mg

Alpha-1 Antitrypsin (AAT), is an inhibitor of the serine protease trypsin. AAT can inhibit many other serine proteases as well, and is thus a general serine protease inhibitor or serpin1. The first part of its name comes from the fact that AAT is a protein that migrates in the alpha-1 region on serum protein electrophoresis2.

AAT is produced by the liver and is one of the classical acute phase response proteins, with serum levels increasing during states of acute inflammation3-6. The role of AAT in most circumstances is protective. As a protease inhibitor, its main role is to prevent undesired proteolytic degradation7,8,9. It is particularly important in inhibiting neutrophil elastase in the lung, and the absence or deficiency of this inhibition leads to morbidity and mortality in a variety of respiratory conditions10-14. AAT can also act as an anti-inflammatory agent and can directly modulate the immune response in various ways, effecting the proliferation of lymphocytes and the function of other immune system effectors such as monocytes, neutrophils and cytokines such as TNF-alpha15-18.

The measurement of serum AAT levels can be clinically useful for a variety of conditions, the most obvious one being AAT deficiency, a disease usually caused by any one of a number of genetic mutations19. AAT deficiency can result in early severe emphysema11,12,20 and cirrhosis and other diseases of the liver20,21, due to uninhibited proteolytic activity in these tissues. The measurement of AAT and various AAT complexes has also been reported to be useful in the diagnosis, prognosis and in monitoring of other specific pathologies22-27, though few have gained widespread use.

ADULT ALPHA-1 ANTITRYPSIN REFERENCE RANGE:90-200 mg/dL

REFERENCES
  1. 1. Burtis, C.A., Ashwood, E.R. and Bruns, E.R. Tietz Textbook of Clinical Chemistry, 4th Edition, W.B. Saunders Company, 2006.
  2. 2. Sun, T. Interpretation of Protein and Isoenzyme Patterns in Body Fluids. Igaku-Shoin Medical Publishers Inc., 1991.
  3. 3. Romette, J. et. al. Inflammatory Syndrome and Changes in Plasma Proteins, Pathologie-Biologie (Paris), 34, 1006-1012, 1986.
  4. 4. Engler, R. Proteins of the Inflammatory Reaction. Regulatory Functions. Ann Biol Clin (Paris), 46, 336-342, 1986.
  5. 5. Stahl, W. Acute Phase Protein Response to Tissue Injury. Critical Care Medicine, 15, 545-550, 1987.
  6. 6. Calvin, J., et. al. The Relative Merits of Acute Phase Proteins in the Recognition of Inflammatory Conditions. Ann Clin Biochem, 25, 60-66, 1988.
  7. 7. Brower, M.S. and Harpel, P.C. Alpha-1-Antitrypsin-Human Leukocyte Elastase Complexes in Blood: Quantification by an Enzyme-Linked Differential Antibody Immunosorbent Assay and Comparison with Alpha-2-Plasmin Inhibitor-Plasmin Complexes. Blood, 61, 842-849, 1983.
  8. 8. Carrell, R.W. Alpha 1-Antitrypsin: Molecular Pathology, Leukocytes, and Tissue Damage. Journal of Clinical Investigation, 78, 1427-1431, 1986.
  9. 9. Sun, Z. and Yang, P. Role of Imbalance Between Neutrophil Elastase and α1-Antitrypsin in Cancer Development and Progression. The Lancet Oncology, 5, 182-190, 2004.
  10. 10. Morrison, H.M., et. al. Inhibitory Capacity of Alpha 1 Antitrypsin in Lung Secretions: Variability and the Effect of Drugs. Thorax, 39, 510-516, 1984.
  11. 11. Brantly, M.L., et. al. Clinical Features and History of the Destructive Lung Disease Associated with Alpha-1-Antitrypsin Deficiency of Adults with Pulmonary Symptoms. Ann Rev Respir Dis, 38, 327-336, 1988.
  12. 12. Pierce, J.A. Antitrypsin and Emphysema. JAMA, 259, 2890-2895, 1988.
  13. 13. McElvaney, N.G., et. al. Aerosol α1-Antitrypsin Treatment for Cystic Fibrosis. The Lancet, 337, 392-394. 1991.
  14. 14. Stoller, J.K. and Aboussouan, L.S. α1-Antitrypsin Deficiency. The Lancet, 365, 2225-2236, 2005.
  15. 15. Breit, S.N., et al. The Role of α1-Antitrypsin Deficiency in the Pathogenesis of Immune Disorders. Clin Immunol Immunopathol, 35, 363-380, 1985.
  16. 16. Van Molle, W., et. al. Alpha 1 Acid Glycoprotein and Alpha 1-Antitrypsin Inhibit TNF-Induced but not anti-Fas-Induced Apoptosis of Hepatocytes in Mice. Journal of Immunology, 159, 3555-3564, 1997.
  17. 17. Churg, A., et. al. Alpha-1-Antitrypsin and a Broad Spectrum Metalloprotease Inhibitor, RS113456, Have Similar Acute Anti-Inflammatory Effects. Laboratory Investigation, 81, 1119-1131, 2001.
  18. 18. Stockley, R.A., et. al. The Effect of Augmentation Therapy on Bronchial Inflammation in α1-Antitrypsin Deficiency. Am J Respir Crit Care Med, 165, 1494-1498, 2002.
  19. 19. Herr, C., et. al. Identification and Analysis of Rare and Novel Mutations of the Alpha-1-Antitrypsin Gene. Am J Respir Crit Care Med, 179, A3502, 2009.
  20. 20. Crystal, R.G. Alpha 1-Antitrypsin Deficiency, Emphysema, and Liver Disease. Genetic Basis and Strategies for Therapy. Journal of Clinical Investigation, 85, 1343-1352, 1990.
  21. 21. Perlmutter, D.H. Alpha-1-Antitrypsin Deficiency: Diagnosis and Treatment. Clinics in Liver Disease, 8, 839-859, 2004.
  22. 22. Laurent, M.R., et. al. Circulating Immune Complexes, Serum Immunoglobulins, and Acute Phase Proteins in Psoriasis and Psoriatic Arthritis. Annals of the Rheumatic Diseases, 40, 66-69, 1981.
  23. 23. Hollsing, A.E., et. al. Granulocyte Elastase-Alpha-1-Antiproteinase Complex in Cystic Fibrosis: Sensitive Plasma Assay for Monitoring Pulmonary Infections. Journal of Pediatrics, 111, 206-211, 1987.
  24. 24. Karashima, S., et. al. Prognostic Significance of Alpha-1-Antitrypsin in Early Stage of Colorectal Carcinomas. International Journal of Cancer, 45, 244-250, 1990.
  25. 25. Higashiyama, M., et. al. An Evaluation of the Prognostic Significance of Alpha-1-Antitrypsin Expression in Adenocarcinoma of the Lung: An Immunohistochemical Analysis. British Cancer Journal, 65, 300-302, 1992.
  26. 26. Hedstrom, J., et. al. Serum Complex of Trypsin 2 and (alpha)(sub1) Antitrypsin as Diagnostic and Prognostic Marker of Acute Pancreatitis: Clinical Study in Consecutive Patients. British Medical Journal, 313, 333, 1996.
  27. 27. Solakidi, S., et. al. Tumour-Associated Trypsin Inhibitor, Carcinoembryonic Antigen and Acute-Phase Reactant Proteins CRP and α1-Antitrypsin in Patients with Gastrointestinal Malignancies. Clinical Biochemistry, 37, 56-60, 2004.

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