丝氨酸蛋白酶抑制剂超家族概述。

An overview of the serpin superfamily.

作者信息

Law Ruby H P, Zhang Qingwei, McGowan Sheena, Buckle Ashley M, Silverman Gary A, Wong Wilson, Rosado Carlos J, Langendorf Chris G, Pike Rob N, Bird Philip I, Whisstock James C

机构信息

Department of Biochemistry and Molecular Biology, Monash University, Clayton Campus, Melbourne VIC 3800, Australia.

出版信息

Genome Biol. 2006;7(5):216. doi: 10.1186/gb-2006-7-5-216. Epub 2006 May 30.

DOI:10.1186/gb-2006-7-5-216

PMID:16737556

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1779521/

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.

摘要

丝氨酸蛋白酶抑制剂（Serpins）是一类广泛分布的蛋白酶抑制剂家族，它们通过构象变化来抑制靶酶。它们在控制许多重要的蛋白水解级联反应中起核心作用，包括哺乳动物的凝血途径。丝氨酸蛋白酶抑制剂的构象不稳定，许多与疾病相关的丝氨酸蛋白酶抑制剂突变会导致错误折叠或形成致病性的无活性聚合物。