By George M. Rodgers
This entire quantity discusses the protease ADAMTS13, summarizing the present prestige of easy and medical learn. The 9 authoritative chapters start with a ancient point of view via exploration of the biochemistry and structure-function relationships of ADAMTS13 in addition to its common functionality in hemostasis (cleavage of von Willebrand factor). rising study topics for ADAMTS13 are lined, together with its capability position in angiogenesis and different points of cellphone biology. extra themes contain laboratory assays for ADAMTS13, inherited ADAMTS13 deficiency, and purchased ADAMTS13 deficiency. A bankruptcy on comparable thrombotic microangiopathic (TMA) problems examines the variations among TMAs linked to ADAMTS13 deficiency and people now not linked to ADAMTS13 deficiency. a last bankruptcy experiences the initial info on rising facets of ADAMTS13, resembling the prestige of recombinant ADAMTS13 items and their power utility.
Comprehensive in its exploration of the ADAMTS13 protease in disease, ADAMTS13: Biology and affliction is an important source for scientific hematologists, transfusion medication physicians, and researchers attracted to hemostasis, vascular biology, biochemistry, and metalloproteases.
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Extra resources for ADAMTS13: Biology and Disease
0100967. Gopalakrishnan K, Kumarasamy S, Abdul-Majeed S, Kalinoski AL, Morgan EE, Gohara AF, Nauli SM, Filipiak WE, Saunders TL, Joe B. Targeted disruption of Adamts16 gene in a rat genetic model of hypertension. Proc Natl Acad Sci U S A. 2012;109(50):20555–9. 1211290109. Jacobi CL, Rudigier LJ, Scholz H, Kirschner KM. Transcriptional regulation by the Wilms tumor protein, Wt1, suggests a role of the metalloproteinase Adamts16 in murine genitourinary development. J Biol Chem. 2013;288(26):18811–24.
Characterization of ADAMTS-9 and ADAMTS-20 as a distinct ADAMTS subfamily related to Caenorhabditis elegans GON-1. J Biol Chem. 2003;278(11):9503–13. M211009200. 11. Koo BH, Le Goff C, Jungers KA, Vasanji A, O’Flaherty J, Weyman CM, Apte SS. ADAMTSlike 2 (ADAMTSL2) is a secreted glycoprotein that is widely expressed during mouse embryogenesis and is regulated during skeletal myogenesis. Matrix Biol. 2007;26(6): 431–41. pii: S0945-053X(07)00042-X. 003. Epub 2007/05/19. 12. Hubmacher D, Apte SS. Genetic and functional linkage between ADAMTS superfamily proteins and fibrillin-1: a novel mechanism influencing microfibril assembly and function.
Arch Pathol Lab Med. 2003;127:834–9. Hunt BJ, Lämmle B, Nevard CHF, Haycock GB, Furlan M. Von Willebrand factor-cleaving protease in childhood diarrhoea-associated haemolytic uraemic syndrome. Thromb Haemost. 2001;85:975–8. Motto DG, Chauhan AK, Zhu G, Homeister J, Lamb CB, Desch KC, Zhang W, Tsai HM, Wagner DD, Ginsburg D. Shigatoxin triggers thrombotic thrombocytopenic purpura in genetically susceptible ADAMTS13-deﬁcient mice. J Clin Invest. 2005;115:2752–61. Chapter 2 Overview of the ADAMTS Superfamily Suneel S.