Gracias por responder. The correct answer is B. The autosomal recessive diseases is alpha-1-antitrypsin deficiency, in which an abnormal form of alpha-1-antitrypsin can be produced by, but not released from, liver cells. This enzyme was named when it was found in vivo to function as a good inhibitor of the pancreatic protease trypsin. However, it actually inactivates a wide variety of enzymes with protease activity that have an essential serine residue in the catalytic centers. Examples of these serine proteases include all of the answer choices, and additionally, cathepsin G and plasminogen. Surprisingly, alpha-1-antitrypsin disease sepcifically targets only the liver and lungs. The liver disease, which can be mild to life-threatening (either due to cirrhosis or, rarely, massive acute hepatic failure), is related to retained alpha-1-antitrypsin with residual enzymatic activity. The lung disease seems to be the specific result of an inability to turn off elastase produced by neutrophils during lung nfections. Neutrophil elastase is one of the few enzymes that can successfully destroy the heavily corsslinked, insoluble elastin that is presented in the lung interstitium and contributes to its elasticity. With destruction of this lattice-like elastic tissue support structure, emphysema can develop in the lungs
Posted on: Mon, 10 Nov 2014 15:40:33 +0000
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