Abstract
Serpins are a broadly distributed family of protease inhibitors that use a conformational change to inhibit target enzymes. They are central in controlling many important proteolytic cascades, including the mammalian coagulation pathways. Serpins are conformationally labile and many of the disease-linked mutations of serpins result in misfolding or in pathogenic, inactive polymers.
Publication types
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Genetic Predisposition to Disease
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Humans
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Mutation
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Protein Conformation
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Serine Proteinase Inhibitors / chemistry*
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Serine Proteinase Inhibitors / classification
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Serine Proteinase Inhibitors / physiology*
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Serpins / chemistry*
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Serpins / classification
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Serpins / physiology*
Substances
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Serine Proteinase Inhibitors
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Serpins