Molecular characterization of ADAMTS13 gene mutations in Japanese patients with Upshaw-Schulman syndrome.
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Pathophysiology of thrombotic thrombocytopenic purpuraPathogenesis of thrombotic microangiopathiesProteolytic processing of von Willebrand factor by adamts13 and leukocyte proteasesThe von Willebrand factor-cleaving protease (ADAMTS-13) and the diagnosis of thrombotic thrombocytopenic purpura (TTP)Identification of strain-specific variants of mouse Adamts13 gene encoding von Willebrand factor-cleaving proteaseAttack of the acronyms: TTP, VWF, ADAMTS-13 and SELDI-TOF-MS.FRETS-VWF73, a first fluorogenic substrate for ADAMTS13 assay.The course of ADAMTS-13 activity and inhibitor titre in the treatment of thrombotic thrombocytopenic purpura with plasma exchange and vincristine.Enzymatically active ADAMTS13 variants are not inhibited by anti-ADAMTS13 autoantibodies: a novel therapeutic strategy?Molecular biology of ADAMTS13 and diagnostic utility of ADAMTS13 proteolytic activity and inhibitor assays.ADAMTS13 and microvascular thrombosisTwo mechanistic pathways for thienopyridine-associated thrombotic thrombocytopenic purpura: a report from the SERF-TTP Research Group and the RADAR Project.The kidney in thrombotic thrombocytopenic purpura.Natural history of Upshaw-Schulman syndrome based on ADAMTS13 gene analysis in Japan.Autopsy case of sudden maternal death from thrombotic thrombocytopenic purpura.Two novel heterozygote missense mutations of the ADAMTS13 gene in a child with recurrent thrombotic thrombocytopenic purpuraTwo newborn-onset patients of Upshaw-Schulman syndrome with distinct subsequent clinical courses.The novel ADAMTS13-p.D187H mutation impairs ADAMTS13 activity and secretion and contributes to thrombotic thrombocytopenic purpura in mice.Pathophysiology of Pediatric Multiple Organ Dysfunction Syndrome.Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) 2017 in Japan.Candidate gene analysis using genomic quantitative PCR: identification of ADAMTS13 large deletions in two patients with Upshaw-Schulman syndrome.Why Do We Need ADAMTS13?Recombinant CUB-1 domain polypeptide inhibits the cleavage of ULVWF strings by ADAMTS13 under flow conditions.The proximal carboxyl-terminal domains of ADAMTS13 determine substrate specificity and are all required for cleavage of von Willebrand factor.The ADAMTS13 metalloprotease domain: roles of subsites in enzyme activity and specificity.Inherited thrombotic thrombocytopenic purpura.Congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) caused by novel ADAMTS13 mutations.Thrombotic thrombocytopenic purpuraThrombotic thrombocytopenic purpura
P2860
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P2860
Molecular characterization of ADAMTS13 gene mutations in Japanese patients with Upshaw-Schulman syndrome.
description
2003 nî lūn-bûn
@nan
2003 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2003 թվականի հոտեմբերին հրատարակված գիտական հոդված
@hy
2003年の論文
@ja
2003年論文
@yue
2003年論文
@zh-hant
2003年論文
@zh-hk
2003年論文
@zh-mo
2003年論文
@zh-tw
2003年论文
@wuu
name
Molecular characterization of ...... with Upshaw-Schulman syndrome.
@ast
Molecular characterization of ...... with Upshaw-Schulman syndrome.
@en
type
label
Molecular characterization of ...... with Upshaw-Schulman syndrome.
@ast
Molecular characterization of ...... with Upshaw-Schulman syndrome.
@en
prefLabel
Molecular characterization of ...... with Upshaw-Schulman syndrome.
@ast
Molecular characterization of ...... with Upshaw-Schulman syndrome.
@en
P2093
P1433
P1476
Molecular characterization of ...... with Upshaw-Schulman syndrome.
@en
P2093
Asayuki Iwai
Etsuro Ito
Fumiaki Banno
Hideaki Umeyama
Hideo Yagi
Hiromichi Ishizashi
Kenji Soejima
Koichi Kokame
Masanori Matsumoto
Masayoshi Miura
P304
P356
10.1182/BLOOD-2003-06-1796
P407
P577
2003-10-16T00:00:00Z