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- W2085205411 startingPage "15" @default.
- W2085205411 abstract "The serpins are the largest superfamily of protease inhibitors. They are found in almost all branches of life including viruses, prokaryotes and eukaryotes. They inhibit their target protease by a unique mechanism that involves a large conformational transition and the translocation of the enzyme from the upper to the lower pole of the protein. This complex mechanism, and the involvement of serpins in important biological regulatory processes, make them prone to mutation-related diseases. For example the polymerization of mutant α 1-antitrypsin leads to the accumulation of ordered polymers within the endoplasmic reticulum of hepatocytes in association with cirrhosis. An identical process in the neuron specific serpin, neuroserpin, results in the accumulation of polymers in neurons and the dementia FENIB. In both cases there is a clear correlation between the molecular instability, the rate of polymer formation and the severity of disease. A similar process underlies the hepatic retention and plasma deficiency of antithrombin, C1 inhibitor, α 1-antichymotrypsin and heparin co-factor II. The common mechanism of polymerization has allowed us to group these conditions together as a novel class of disease, the serpinopathies." @default.
- W2085205411 created "2016-06-24" @default.
- W2085205411 creator A5015134008 @default.
- W2085205411 creator A5036734448 @default.
- W2085205411 creator A5061321693 @default.
- W2085205411 creator A5074710700 @default.
- W2085205411 date "2007-01-01" @default.
- W2085205411 modified "2023-10-16" @default.
- W2085205411 title "Protein Misfolding and the Serpinopathies" @default.
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