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ADAMTS13 pp 21–37Cite as

Overview of the ADAMTS Superfamily

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Abstract

ADAMTS13 is an idiosyncratic member of a distinct family of zinc metalloproteases, the ADAMTS family. This 19-member family is related to ADAM proteases and matrix metalloproteinases (MMPs) through sequence homology of their active sites and similar catalytic domain structures. These similarities contrast with the presence of distinct ancillary domains in each family, with those of ADAMTS proteases comprising thrombospondin type 1 repeats and other modules in a characteristic arrangement. Human and animal mutations affecting ADAMTS proteases lead to a variety of inherited human disorders and animal phenotypes, which demonstrate their requirement in crucial biological and disease pathways. ADAMTS proteases are implicated in procollagen maturation (ADAMTS2, ADAMTS3), versican turnover during embryogenesis (ADAMTS1, ADAMTS5, ADAMTS9, ADAMTS15, ADAMTS20) and ovulation (ADAMTS1), cartilage aggrecan destruction in arthritis (ADAMTS4, ADAMTS5), genetic disorders affecting fibrillin microfibrils (ADAMTS10, ADAMTS17), and, recently, VEGF-C processing during lymphangiogenesis (ADAMTS3). Although ADAMTS13 shares many characteristics with the other family members, it has some special attributes and is the only ADAMTS protease presently implicated in hemostasis. Its unique specificity for von Willebrand factor contrasts with that of other family members, which can have shared or multiple substrates.

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  1. Department of Biomedical Engineering (ND20), Lerner Research Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH, 44195, USA

    Suneel S. Apte M.B.B.S., D.Phil.

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  1. Division of Hematology, University of Utah Health Sciences Center, Salt Lake City, Utah, USA

    George M. Rodgers

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Apte, S.S. (2015). Overview of the ADAMTS Superfamily. In: Rodgers, G. (eds) ADAMTS13. Springer, Cham. https://doi.org/10.1007/978-3-319-08717-7_2

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