ADAMTS13: Biology and Disease - download pdf or read online

By George M. Rodgers

ISBN-10: 3319087169

ISBN-13: 9783319087160

ISBN-10: 3319087177

ISBN-13: 9783319087177

This accomplished quantity discusses the protease ADAMTS13, summarizing the present prestige of uncomplicated and medical examine. The 9 authoritative chapters start with a historic standpoint via exploration of the biochemistry and structure-function relationships of ADAMTS13 in addition to its general functionality in hemostasis (cleavage of von Willebrand factor). rising study subject matters for ADAMTS13 are coated, together with its strength position in angiogenesis and different elements of telephone biology. extra themes contain laboratory assays for ADAMTS13, inherited ADAMTS13 deficiency, and bought ADAMTS13 deficiency. A bankruptcy on comparable thrombotic microangiopathic (TMA) issues examines the variations among TMAs linked to ADAMTS13 deficiency and people now not linked to ADAMTS13 deficiency. a last bankruptcy reports the initial info on rising features of ADAMTS13, comparable to the prestige of recombinant ADAMTS13 items and their strength utility.

Comprehensive in its exploration of the ADAMTS13 protease in disease, ADAMTS13: Biology and sickness is an important source for medical hematologists, transfusion medication physicians, and researchers drawn to hemostasis, vascular biology, biochemistry, and metalloproteases.

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A genetic defect of connective tissues. J Hered. 1974;65(6):356–8. 71. Lapiere CM, Lenaers A, Kohn LD. Procollagen peptidase: an enzyme excising the coordination peptides of procollagen. Proc Natl Acad Sci U S A. 1971;68(12):3054–8. 72. Nusgens BV, Verellen-Dumoulin C, Hermanns-Le T, De Paepe A, Nuytinck L, Pierard GE, Lapiere CM. Evidence for a relationship between Ehlers-Danlos type VII C in humans and bovine dermatosparaxis. Nat Genet. 1992;1(3):214–7. S. Apte 73. Colige A, Sieron AL, Li SW, Schwarze U, Petty E, Wertelecki W, Wilcox W, Krakow D, Cohn DH, Reardon W, Byers PH, Lapiere CM, Prockop DJ, Nusgens BV.

They lack the regions comprising the protease domain of ADAMTS proteases (Fig. 1), and, therefore, are not proteases [7]. S. Phil. M. S. Apte Protease domain Ancillary domain ADAMTS4 _ Signal peptide ADAMTS5/8 1 Propeptide ADAMTS1/15 2 Catalytic module ADAMTS9/20 14 TSR (central) ADAMTS6/10 4 Cysteine-rich module ADAMTS17/19 4 ADAMTS2/3/14 3 ADAMTS7/12 3 ADAMTS16/18 5 ADAMTS13 6 Disintegrin-like module Spacer n TSR (C-terminal) PLAC Shared modules Procollagen N-propeptidase CUB Mucin/proteoglycan Proteoglycanases Gon-1 related Procollagen aminopropeptidases 4 Mucin-proteoglycans vWF-protease Gon-1 Clade-specific modules Fig.

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. 464644. Aldahmesh MA, Alshammari MJ, Khan AO, Mohamed JY, Alhabib FA, Alkuraya FS. The syndrome of microcornea, myopic chorioretinal atrophy, and telecanthus (MMCAT) is caused by mutations in ADAMTS18. Hum Mutat. 2013;34(9):1195–9. 22374. Peluso I, Conte I, Testa F, Dharmalingam G, Pizzo M, Collin RW, Meola N, Barbato S, Mutarelli M, Ziviello C, Barbarulo AM, Nigro V, Melone MA, European Retinal Disease C, Simonelli F, Banfi S.

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ADAMTS13: Biology and Disease by George M. Rodgers

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