| 1 |
35491445 | VWF-Gly2752Ser, a novel non-cysteine substitution variant in the CK domain, exhibits severe secretory impairment by hampering C-terminal dimer formation. | 2022 May 1 |
2 |
| 2 |
34105490 | [Biological Function of CysR Domain of ADAMTS13]. | 2021 Jun |
2 |
| 3 |
28533135 | Enhanced Local Disorder in a Clinically Elusive von Willebrand Factor Provokes High-Affinity Platelet Clumping. | 2017 Jul 7 |
2 |
| 4 |
26581637 | Free thiol groups in von Willebrand factor (VWF) are required for its full function under physiological flow conditions. | 2016 Jan |
2 |
| 5 |
26670633 | von Willebrand factor is dimerized by protein disulfide isomerase. | 2016 Mar 3 |
1 |
| 6 |
25564400 | The role of the ADAMTS13 cysteine-rich domain in VWF binding and proteolysis. | 2015 Mar 19 |
5 |
| 7 |
25690668 | von Willebrand factor arginine 1205 substitution results in accelerated macrophage-dependent clearance in vivo. | 2015 May |
1 |
| 8 |
25792731 | ADAMTS13, lucky to have a hydrophobic pocket. | 2015 Mar 19 |
2 |
| 9 |
24283831 | The von Willebrand factor predicted unpaired cysteines are essential for secretion. | 2014 Feb |
8 |
| 10 |
25230768 | Loss of cysteine 584 impairs the storage and release, but not the synthesis of von Willebrand factor. | 2014 Dec |
4 |
| 11 |
23446343 | C2362F mutation gives rise to an ADAMTS13-resistant von Willebrand factor. | 2013 Jun |
2 |
| 12 |
23539537 | Insights into pathological mechanisms of missense mutations in C-terminal domains of von Willebrand factor causing qualitative or quantitative von Willebrand disease. | 2013 Aug |
4 |
| 13 |
22207689 | Biogenesis of Weibel-Palade bodies in von Willebrand's disease variants with impaired von Willebrand factor intrachain or interchain disulfide bond formation. | 2012 Jun |
3 |
| 14 |
22102189 | The international society on thrombosis and haematosis von Willebrand disease database: an update. | 2011 Jul |
1 |
| 15 |
20351307 | A cluster of mutations in the D3 domain of von Willebrand factor correlates with a distinct subgroup of von Willebrand disease: type 2A/IIE. | 2010 Jun 10 |
1 |
| 16 |
20354169 | The importance of vicinal cysteines, C1669 and C1670, for von Willebrand factor A2 domain function. | 2010 Jun 10 |
3 |
| 17 |
20570857 | The dominant-negative von Willebrand factor gene deletion p.P1127_C1948delinsR: molecular mechanism and modulation. | 2010 Dec 9 |
1 |
| 18 |
19506352 | Laboratory diagnosis and molecular classification of von Willebrand disease. | 2009 |
1 |
| 19 |
19506359 | Laboratory diagnosis of von Willebrand disease type 1/2E (2A subtype IIE), type 1 Vicenza and mild type 1 caused by mutations in the D3, D4, B1-B3 and C1-C2 domains of the von Willebrand factor gene. Role of von Willebrand factor multimers and the von Willebrand factor propeptide/antigen ratio. | 2009 |
6 |
| 20 |
18492952 | Extensive contacts between ADAMTS13 exosites and von Willebrand factor domain A2 contribute to substrate specificity. | 2008 Sep 1 |
1 |
| 21 |
17118332 | Mapping von Willebrand factor A domain binding sites on a snake venom metalloproteinase cysteine-rich domain. | 2007 Jan 1 |
8 |
| 22 |
17119126 | Effect of von Willebrand factor Y/C1584 on in vivo protein level and function and interaction with ABO blood group. | 2007 Apr 1 |
2 |
| 23 |
17296575 | The association between the L1565 variant of von Willebrand factor and susceptibility to proteolysis by ADAMTS13. | 2007 Feb |
3 |
| 24 |
17393013 | Altered von Willebrand factor subunit proteolysis and multimer processing associated with the Cys2362Phe mutation in the B2 domain. | 2007 Apr |
1 |
| 25 |
17578514 | Interaction of the cysteine-rich domain of snake venom metalloproteinases with the A1 domain of von Willebrand factor promotes site-specific proteolysis of von Willebrand factor and inhibition of von Willebrand factor-mediated platelet aggregation. | 2007 Jul |
4 |
| 26 |
17610557 | C1584 in von Willebrand factor is necessary for enhanced proteolysis by ADAMTS13 in vitro. | 2007 Jul |
4 |
| 27 |
17895385 | Two Cys residues essential for von Willebrand factor multimer assembly in the Golgi. | 2007 Oct 2 |
3 |
| 28 |
16428330 | Analysis on the molecular species and concentration of circulating ADAMTS13 in Blood. | 2006 Jan |
1 |
| 29 |
17139364 | Differential effects of the loss of intrachain- versus interchain-disulfide bonds in the cystine-knot domain of von Willebrand factor on the clinical phenotype of von Willebrand disease. | 2006 Dec |
3 |
| 30 |
15662617 | Interplay between ADAMTS13 and von Willebrand factor in inherited and acquired thrombotic microangiopathies. | 2005 Jan |
1 |
| 31 |
15929722 | Function of the cysteine-rich domain of the haemorrhagic metalloproteinase atrolysin A: targeting adhesion proteins collagen I and von Willebrand factor. | 2005 Oct 1 |
4 |
| 32 |
16194200 | Cysteine-mutations in von Willebrand factor associated with increased clearance. | 2005 Oct |
3 |
| 33 |
14995987 | Dimerization and multimerization defects of von Willebrand factor due to mutated cysteine residues. | 2004 Feb |
4 |
| 34 |
15213842 | Recombinant expression of mutations causing von Willebrand disease type Normandy: characterization of a combined defect of factor VIII binding and multimerization. | 2004 Jul |
1 |
| 35 |
15461624 | Expression of two type 2N von Willebrand disease mutations identified in exon 18 of von Willebrand factor gene. | 2004 Oct |
1 |
| 36 |
15579080 | Structural basis and therapeutic implication of the interaction of CCN proteins with glycoconjugates. | 2004 |
1 |
| 37 |
15620212 | Amino acid sequence of alpha-subunit in hen egg white ovomucin deduced from cloned cDNA. | 2004 Aug |
1 |
| 38 |
12176890 | The role of the D1 domain of the von Willebrand factor propeptide in multimerization of VWF. | 2002 Sep 1 |
2 |
| 39 |
11150026 | Type 2M von Willebrand disease variant characterized by abnormal von willebrand factor multimerization. | 2001 Jan |
2 |
| 40 |
11159522 | The arginine-552-cysteine (R1315C) mutation within the A1 loop of von Willebrand factor induces an abnormal folding with a loss of function resulting in type 2A-like phenotype of von Willebrand disease: study of 10 patients and mutated recombinant von Willebrand factor. | 2001 Feb 15 |
2 |
| 41 |
11468166 | Aberrant dimerization of von Willebrand factor as the result of mutations in the carboxy-terminal region: identification of 3 mutations in members of 3 different families with type 2A (phenotype IID) von Willebrand disease. | 2001 Aug 1 |
1 |
| 42 |
10807780 | Conformational changes in the D' domain of von Willebrand factor induced by CYS 25 and CYS 95 mutations lead to factor VIII binding defect and multimeric impairment. | 2000 May 15 |
2 |
| 43 |
10831592 | Localization of disulfide bonds in the cystine knot domain of human von Willebrand factor. | 2000 Aug 18 |
4 |
| 44 |
10196713 | Mutation in the zonadhesin-like domain of alpha-tectorin associated with autosomal dominant non-syndromic hearing loss. | 1999 Feb-Mar |
1 |
| 45 |
10477273 | Production and functional activity of a recombinant von Willebrand factor-A domain from human complement factor B. | 1999 Sep 15 |
1 |
| 46 |
9164870 | Molecular cloning of a major human gall bladder mucin: complete C-terminal sequence and genomic organization of MUC5B. | 1997 May 15 |
1 |
| 47 |
8622978 | Defective dimerization of von Willebrand factor subunits due to a Cys-> Arg mutation in type IID von Willebrand disease. | 1996 Apr 16 |
1 |
| 48 |
8839833 | Dominant type 1 von Willebrand disease caused by mutated cysteine residues in the D3 domain of von Willebrand factor. | 1996 Oct 1 |
7 |
| 49 |
7539426 | Identification of amino acid residues essential for von Willebrand factor binding to platelet glycoprotein Ib. Charged-to-alanine scanning mutagenesis of the A1 domain of human von Willebrand factor. | 1995 Jun 2 |
2 |
| 50 |
8578449 | Structure-function relationship of the A1 domain of von Willebrand factor. | 1995 Jul |
1 |