Aim of this study was to analyse the performance of the CliA ADAMTS13 activity assay in detecting inhibitory ADAMTS13 antibodies using the Bethesda assay.Montaruli, BarbaraNovelli, ChiaraSolfietti, LauraValpreda, AlessandraBazzan, MarioLuigi Andrea Beverina, IvoBrando, BrunoRoccatello, DarioCosseddu...
显示出溶血性贫血、血小板减少、肾功能障碍等作为血栓性微小血管障碍症(thromboticmicroangiopathy;tma)的病理所见的3个症候,但对患者进行活体肝移植而生长时,上述tma所见消失(matsumotom,chisuwah,nakazaway,等人:livertransplantationrescuesadeficientstateofvonwillebrandfactorcleavingproteaseactivityinpatientswithliver...
2003. ADAMTS-13 gene mutation In congenital thrombotic thrombocytopenic purpura with previously reported normal VWF cleaving protease activity. Blood 101:4449–4451. Article PubMed CAS Google Scholar Schneppenheim, R., Budde, U., Oyen, F., Angerhaus, D., Aumann, V., Drewke, E., Hassen...
在治疗的血浆制品中ADAMTS13活性的比较和稳定性 在治疗的血浆制品中ADAMTS13活性的比 较和稳定性 国际输血及血液学杂志2007年堂箜 DavevFR,Ols0nS,KurecAS,eta1.Theimmun0phen0typing0f extramedullarymyeloidcelltumorsinparaffinembeddedtissue sections.AmJSurgPathol,1988,12(9):699—707.BrunningRD,BennettJ,...
ADAMTS13 that was truncated after the metalloprotease domain, the disintegrin domain, the first TSP1 repeat, or the Cys-rich domain was not able to cleave von Willebrand factor, whereas addition of the spacer region restored protease activity. Therefore, the spacer region is necessary for normal ...
ADAMTS13, a disintegrin and metalloprotease with a thrombospondin type 1 motif, member 13, regulates the length of Von Willebrand factor (VWF) multimers and their platelet-binding activity. ADAMTS13 is constitutively secreted as an active protease and is not inhibited by circulating protease inhibitors...
UL‑vWF与TTP之间的关 联随着由Furlan等以及Tsai和Lian独立发现大多数罹患TTP的患者缺乏裂解vWF的血浆金 属蛋白酶(现已知为ADAMTS13)而得到支持(Furlan M,Robles R,Solenthaler M,Wassmer M,Sandoz P,Laemmle B.Deficient activity of von Willebrand factor‑cleaving protease in chronic relapsing thrombotic ...
健康成人中的血浆ADAMTS13活性在50%至178%的范围内(MoakeJL.Thromboticthrombocytopenicpurpuraandthehemolyticuremicsyndrome.ArchPatholLabMed.2002;126:1430-1433)在大多数患有家族性或获得性TTP的患者中,血浆ADAMTS13活性不存在或小于正常值的5%。在不治疗下,死亡率超过90%,但血楽疗法已将死亡率降至约20%(MoakeJL....
Igari A, Nakagawa T, Moriki T, et al. Identification of epitopes on ADAMTS13 recognized by a panel of monoclonal antibodies with functional or non-functional effects on catalytic activity. Thromb Res. 2012;130:e79–83. doi:10.1016/j.thromres.2012.06.006. ...
A Comparison of Two Commercial ADAMTS13 Activity Assays With a Reference Laboratory Method Crist RA, Rodgers GM. A comparison of two commercial ADAMTS13 activity assays with a reference laboratory method. Lab Med. 2009;40(4):232- 235... RA Crist,GM Rodgers - 《Laboratory Medicine》 被引量:...