An overview of the serpin superfamily
1 Department of Biochemistry and Molecular Biology, Monash University, Clayton Campus, Melbourne VIC 3800, Australia
2 Victorian Bioinformatics Consortium, Monash University, Clayton Campus, Melbourne VIC 3800, Australia
3 ARC Centre for Structural and Functional Microbial Genomics, Monash University, Clayton Campus, Melbourne VIC 3800, Australia
4 Magee-Womens Research Institute, Children's Hospital of Pittsburgh, Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA
Genome Biology 2006, 7:216 doi:10.1186/gb-2006-7-5-216Published: 30 May 2006
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.