By George M. Rodgers
This complete quantity discusses the protease ADAMTS13, summarizing the present prestige of uncomplicated and medical study. The 9 authoritative chapters commence 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 examine issues for ADAMTS13 are coated, together with its strength position in angiogenesis and different points of cellphone biology. extra issues 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 stories the initial details on rising features of ADAMTS13, similar to the prestige of recombinant ADAMTS13 items and their power utility.
Comprehensive in its exploration of the ADAMTS13 protease in disease, ADAMTS13: Biology and sickness is an important source for medical hematologists, transfusion drugs physicians, and researchers drawn to hemostasis, vascular biology, biochemistry, and metalloproteases.
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Additional resources for ADAMTS13: Biology and Disease
Morales J, Al-Sharif L, Khalil DS, Shinwari JM, Bavi P, Al-Mahrouqi RA, Al-Rajhi A, Alkuraya FS, Meyer BF, Al Tassan N. Homozygous mutations in ADAMTS10 and ADAMTS17 cause lenticular myopia, ectopia lentis, glaucoma, spherophakia, and short 2 Overview of the ADAMTS Superfamily 88. 89. 90. 91. 92. 93. 94. 95. 96. 97. 98. 99. 100. 101. 102. 35 stature. Am J Hum Genet. 2009;85(5):558–68. pii: S0002-9297(09)00407-8. 011. Epub 2009/10/20. Abdul-Majeed S, Mell B, Nauli SM, Joe B. Cryptorchidism and infertility in rats with targeted disruption of the Adamts16 locus.
014. Epub 2008/09/09. 21. Liu YJ, Xu Y, Yu Q. Full-length ADAMTS-1 and the ADAMTS-1 fragments display pro- and antimetastatic activity, respectively. Oncogene. 2006;25(17):2452–67. 22. Koo BH, Apte SS. Cell-surface processing of the metalloprotease pro-ADAMTS9 is influenced by the chaperone GRP94/gp96. J Biol Chem. 2010;285(1):197–205. 039677. 039677. Epub 2009/10/31. 2 Overview of the ADAMTS Superfamily 31 23. Koo BH, Longpre JM, Somerville RP, Alexander JP, Leduc R, Apte SS. Regulation of ADAMST9 secretion and enzymatic activity by its propeptide.
Genome-wide association studies and transcriptome analysis have identified associations of ADAMTS loci with several common disorders [101–105], but these associations remain suggestive until functionally validated. , with few exceptions, they are not within the exons of the gene, and do not introduce amino acid changes in the proteins. Furthermore, proximity of the SNP to the intergenic or intronic regions of an ADAMTS gene locus does not imply that it is necessarily in a regulatory region of that gene, since the SNP may actually affect regulation of another locus in the general region or, sometimes, even further away if it lies within an enhancer.