Abstract
In plasma, the size of the von Willebrand factor (VWF) multimer is down-regulated by ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13). The binding of platelets or glycoprotein (GP) Ibα recombinant fragment to VWF domain A1 may increase the cleavage by ADAMTS13 to VWF. Both type 2B and type 2M von Willebrand disease (VWD) result in bleeding disorders with the diathesis of increased and decreased binding affinity between GPIbα and VWF, respectively. However, the influence of 2B/2M VWD mutations in the A1 domain and GPIbα on cleavage by ADAMTS13 to VWF needs further study. Different types of full-length human recombinant VWF (rVWF) were expressed, including three type 2B mutations (P1337L, H1268D, and R1308C), one type 2M mutation (D1302G), and wild type (WT). The three characterized types of rVWF were digested by ADAMTS13 under static conditions or high-shear stress. The interaction of rVWF and ADAMTS13 was also tested by plate-binding assays. Under static (natured) conditions or high-shear stress, type 2B mutants exhibited a higher susceptibility to ADAMTS13 than rVWF-WT, whereas type 2M mutant was normal. While under static (denatured) conditions or high-shear stress (with GPIbα fragmen...Continue Reading
References
Apr 1, 1991·The Journal of Clinical Investigation·K A CooneyD Ginsburg
Jan 25, 1991·Cell·D D WagnerT N Mayadas
Apr 1, 1991·The Journal of Clinical Investigation·A M RandiJ E Sadler
Jun 1, 1991·Mayo Clinic Proceedings·H R GralnickR I Parker
Jun 3, 1986·Biochemistry·B B Shelton-InloesJ E Sadler
Sep 2, 1998·American Journal of Hematology·P CasañaJ A Aznar
Dec 29, 2000·Platelets·N CauwenberghsH Deckmyn
Mar 30, 2001·The Journal of Biological Chemistry·G D EnglundX Du
Sep 6, 2001·Blood·K FujikawaD Chung
Oct 5, 2001·Nature·G G LevyH M Tsai
Jan 14, 2004·Journal of Thrombosis and Haemostasis : JTH·Z M Ruggeri
Apr 2, 2005·British Journal of Haematology·Koichi KokameToshiyuki Miyata
Aug 17, 2005·Journal of Thrombosis and Haemostasis : JTH·J E Sadler
Sep 6, 2005·Blood·Zhenyin TaoJing-Fei Dong
Dec 3, 2005·Blood·Wolf Achim HassenpflugReinhard Schneppenheim
Nov 8, 2006·Journal of Thrombosis and Haemostasis : JTH·J RayesJ P Girma
Sep 29, 2007·Blood·Kyuhwan ShimJ Evan Sadler
May 22, 2008·Proceedings of the National Academy of Sciences of the United States of America·Wenjing CaoX Long Zheng
May 22, 2008·Blood·Weiqiang GaoJ Evan Sadler
Jun 10, 2009·Acta Haematologica·Alain GadisseurJan Jacques Michiels
Jul 10, 2009·Blood·Sara ZanardelliDavid A Lane
Jan 16, 2010·Blood·Sheng-Yu JinX Long Zheng
Jul 8, 2010·The Journal of Biological Chemistry·Christopher G SkipwithX Long Zheng
Jan 25, 2012·European Journal of Haematology·Yiming ZhaoChanggeng Ruan
Jul 17, 2013·Journal of Thrombosis and Haemostasis : JTH·C CasariC V Denis
Dec 10, 2013·Hematology·James T B Crawley, Marie A Scully
Jun 15, 2014·Blood·Timothy A Springer