Alpha-1 Antitrypsin: Its Role in Health and Disease
Alpha-1 Antitrypsin (AAT) is a 52 kDa glycoprotein that is principally synthesized by the liver. It is the archetype of the serine protease inhibitor (Serpin) superfamily of proteins, which has a major role in inactivating neutrophil elastase and other proteases to retain protease – antiprotease equilibrium. AAT deficiency is a rare monogenic disorder characterized by low levels of AAT in serum and the lungs and it is well known to be associated with emphysema and liver disease. Inadequate knowledge of AAT deficiency might be due to under-recognition of this protein. To date, the exact role of AAT deficiency in various diseases has not been extensively elucidated. In this review, the current knowledge regarding the role AAT in various disorders will be discussed.
Keywords: Alpha 1-antitrypsin, serine protease inhibitors, SERPIN, Alpha-1 Antitrypsin (AAT), protease inhibitor (Serpin), emphysema, granulocytes, collagenase, proteinase 3, metalloproteinases, elastase, trypsin, alcohol dehydrogenase, aldolase, carbonic anhydrase, catalase, citrate synthase, enolase, glutathione S-transferase, l-lactate dehydrogenase, beta (L)-crystallin, pleomorphic gene, single nucleotide polymorphisms (SNPs), glutamic acid to lysine at position 342 (Glu342Lys), immunohistochemical staining, augmentation therapy, dysfunctional alleles, chronic bronchitis, bronchiectasis, neonatal jaundice, liver cirrhosis, vasculitis, panniculitis, Computerized tomography (CT), hepatocellular carcinoma, reactive oxygen species, psoriasis, lymphokine secretion, eicosanoid metabolism, polymorphonuclear leucocytes, psoriatic fibroblasts, serum trypsin inhibitory capacity (sTIC), chloramines, peroxynitrite, necrosis, serosanguinous fluid, hepatoma, Wegener's granulomatosis, rheumatoid arthritis, blepharitis, keratitis, uveitis, eczema, Hemodialysis (HD), chronic rhinosinusitis (CRS)
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