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Crystal structure of the von Willebrand Factor A1 domain and implications for the binding of platelet glycoprotein Ib.

Emsley J, Cruz M, Handin R, Liddington R
J Biol Chem 273 10396-401 (1998)
Cited: 171 times
EuropePMC logo PMID: 9553097

Abstract

von Willebrand Factor (vWF) is a multimeric protein that mediates platelet adhesion to exposed subendothelium at sites of vascular injury under conditions of high flow/shear. The A1 domain of vWF (vWF-A1) forms the principal binding site for platelet glycoprotein Ib (GpIb), an interaction that is tightly regulated. We report here the crystal structure of the vWF-A1 domain at 2.3-A resolution. As expected, the overall fold is similar to that of the vWF-A3 and integrin I domains. However, the structure also contains N- and C-terminal arms that wrap across the lower surface of the domain. Unlike the integrin I domains, vWF-A1 does not contain a metal ion-dependent adhesion site motif. Analysis of the available mutagenesis data suggests that the activator botrocetin binds to the right-hand face of the domain containing helices alpha5 and alpha6. Possible binding sites for GpIb are the front and upper surfaces of the domain. Natural mutations that lead to constitutive GpIb binding (von Willebrand type IIb disease) cluster in a different site, at the interface between the lower surface and the terminal arms, suggesting that they disrupt a regulatory region rather than forming part of the primary GpIb binding site. A possible pathway for propagating structural changes from the regulatory region to the ligand-binding surface is discussed.

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Articles - 1auq mentioned but not cited (40)

  1. Protein-protein docking benchmark version 3.0. Hwang H, Pierce B, Mintseris J, Janin J, Weng Z. Proteins 73 705-709 (2008)
  2. PyCogent: a toolkit for making sense from sequence. Knight R, Maxwell P, Birmingham A, Carnes J, Caporaso JG, Easton BC, Eaton M, Hamady M, Lindsay H, Liu Z, Lozupone C, McDonald D, Robeson M, Sammut R, Smit S, Wakefield MJ, Widmann J, Wikman S, Wilson S, Ying H, Huttley GA. Genome Biol 8 R171 (2007)
  3. Crucial role for the VWF A1 domain in binding to type IV collagen. Flood VH, Schlauderaff AC, Haberichter SL, Slobodianuk TL, Jacobi PM, Bellissimo DB, Christopherson PA, Friedman KD, Gill JC, Hoffmann RG, Montgomery RR, Zimmerman Program Investigators. Blood 125 2297-2304 (2015)
  4. Predicting interaction sites from the energetics of isolated proteins: a new approach to epitope mapping. Scarabelli G, Morra G, Colombo G. Biophys J 98 1966-1975 (2010)
  5. Structural basis of regulation of von Willebrand factor binding to glycoprotein Ib. Blenner MA, Dong X, Springer TA. J Biol Chem 289 5565-5579 (2014)
  6. Critical von Willebrand factor A1 domain residues influence type VI collagen binding. Flood VH, Gill JC, Christopherson PA, Bellissimo DB, Friedman KD, Haberichter SL, Lentz SR, Montgomery RR. J Thromb Haemost 10 1417-1424 (2012)
  7. N-terminal flanking region of A1 domain in von Willebrand factor stabilizes structure of A1A2A3 complex and modulates platelet activation under shear stress. Auton M, Sowa KE, Behymer M, Cruz MA. J Biol Chem 287 14579-14585 (2012)
  8. Structural Insights into Mdn1, an Essential AAA Protein Required for Ribosome Biogenesis. Chen Z, Suzuki H, Kobayashi Y, Wang AC, DiMaio F, Kawashima SA, Walz T, Kapoor TM. Cell 175 822-834.e18 (2018)
  9. The mechanism of VWF-mediated platelet GPIbalpha binding. Auton M, Zhu C, Cruz MA. Biophys J 99 1192-1201 (2010)
  10. The catch bond mechanism between von Willebrand factor and platelet surface receptors investigated by molecular dynamics simulations. Interlandi G, Thomas W. Proteins 78 2506-2522 (2010)
  11. Activation of von Willebrand factor via mechanical unfolding of its discontinuous autoinhibitory module. Arce NA, Cao W, Brown AK, Legan ER, Wilson MS, Xu ER, Berndt MC, Emsley J, Zhang XF, Li R. Nat Commun 12 2360 (2021)
  12. Protein-protein binding site identification by enumerating the configurations. Guo F, Li SC, Wang L, Zhu D. BMC Bioinformatics 13 158 (2012)
  13. Binding interface prediction by combining protein-protein docking results. Hwang H, Vreven T, Weng Z. Proteins 82 57-66 (2014)
  14. A mechanism for localized dynamics-driven affinity regulation of the binding of von Willebrand factor to platelet glycoprotein Ibα. Liu G, Fang Y, Wu J. J Biol Chem 288 26658-26667 (2013)
  15. N-linked glycan stabilization of the VWF A2 domain. Lynch CJ, Lane DA. Blood 127 1711-1718 (2016)
  16. A small molecule glycosaminoglycan mimetic blocks Plasmodium invasion of the mosquito midgut. Mathias DK, Pastrana-Mena R, Ranucci E, Tao D, Ferruti P, Ortega C, Staples GO, Zaia J, Takashima E, Tsuboi T, Borg NA, Verotta L, Dinglasan RR, Dinglasan RR. PLoS Pathog 9 e1003757 (2013)
  17. A network-based multi-target computational estimation scheme for anticoagulant activities of compounds. Li Q, Li X, Li C, Chen L, Song J, Tang Y, Xu X. PLoS One 6 e14774 (2011)
  18. Minimal ensembles of side chain conformers for modeling protein-protein interactions. Beglov D, Hall DR, Brenke R, Shapovalov MV, Dunbrack RL, Kozakov D, Vajda S. Proteins 80 591-601 (2012)
  19. Structure of the micronemal protein 2 A/I domain from Toxoplasma gondii. Tonkin ML, Grujic O, Pearce M, Crawford J, Boulanger MJ. Protein Sci 19 1985-1990 (2010)
  20. Enhanced Local Disorder in a Clinically Elusive von Willebrand Factor Provokes High-Affinity Platelet Clumping. Tischer A, Machha VR, Frontroth JP, Brehm MA, Obser T, Schneppenheim R, Mayne L, Walter Englander S, Auton M. J Mol Biol 429 2161-2177 (2017)
  21. Glycosylation sterically inhibits platelet adhesion to von Willebrand factor without altering intrinsic conformational dynamics. Tischer A, Machha VR, Moon-Tasson L, Benson LM, Auton M. J Thromb Haemost 18 79-90 (2020)
  22. Structural origins of misfolding propensity in the platelet adhesive von Willebrand factor A1 domain. Zimmermann MT, Tischer A, Whitten ST, Auton M. Biophys J 109 398-406 (2015)
  23. Variable content of von Willebrand factor mutant monomer drives the phenotypic variability in a family with von Willebrand disease. Chen J, Hinckley JD, Haberichter S, Jacobi P, Montgomery R, Flood VH, Wong R, Interlandi G, Chung DW, López JA, Di Paola J. Blood 126 262-269 (2015)
  24. Structures of VWF tubules before and after concatemerization reveal a mechanism of disulfide bond exchange. Anderson JR, Li J, Springer TA, Brown A. Blood 140 1419-1430 (2022)
  25. The structure of the TFIIH p34 subunit reveals a von Willebrand factor A like fold. Schmitt DR, Kuper J, Elias A, Kisker C. PLoS One 9 e102389 (2014)
  26. Identification of a small molecule that modulates platelet glycoprotein Ib-von Willebrand factor interaction. Broos K, Trekels M, Jose RA, Demeulemeester J, Vandenbulcke A, Vandeputte N, Venken T, Egle B, De Borggraeve WM, Deckmyn H, De Maeyer M. J Biol Chem 287 9461-9472 (2012)
  27. The N-terminal autoinhibitory module of the A1 domain in von Willebrand factor stabilizes the mechanosensor catch bond. Zhao YC, Wang H, Wang Y, Lou J, Ju LA. RSC Chem Biol 3 707-720 (2022)
  28. Investigating the clearance of VWF A-domains using site-directed PEGylation and novel N-linked glycosylation. Fazavana J, Brophy TM, Chion A, Cooke N, Terraube V, Cohen J, Parng C, Pittman D, Cunningham O, Lambert M, O'Donnell JS, O'Sullivan JM. J Thromb Haemost 18 1278-1290 (2020)
  29. Evidence for the Misfolding of the A1 Domain within Multimeric von Willebrand Factor in Type 2 von Willebrand Disease. Tischer A, Brehm MA, Machha VR, Moon-Tasson L, Benson LM, Nelton KJ, Leger RR, Obser T, Martinez-Vargas M, Whitten ST, Chen D, Pruthi RK, Bergen HR, Cruz MA, Schneppenheim R, Auton M. J Mol Biol 432 305-323 (2020)
  30. Identification of extant vertebrate Myxine glutinosa VWF: evolutionary conservation of primary hemostasis. Grant MA, Beeler DL, Spokes KC, Chen J, Dharaneeswaran H, Sciuto TE, Dvorak AM, Interlandi G, Lopez JA, Aird WC. Blood 130 2548-2558 (2017)
  31. Determining Complex Structures using Docking Method with Single Particle Scattering Data. Wang H, Liu H. Front Mol Biosci 4 23 (2017)
  32. Exposure of Von Willebrand Factor Cleavage Site in A1A2A3-Fragment under Extreme Hydrodynamic Shear. Languin-Cattoën O, Laborie E, Yurkova DO, Melchionna S, Derreumaux P, Belyaev AV, Sterpone F. Polymers (Basel) 13 3912 (2021)
  33. Noncanonical type 2B von Willebrand disease associated with mutations in the VWF D'D3 and D4 domains. Sacco M, Lancellotti S, Ferrarese M, Bernardi F, Pinotti M, Tardugno M, De Candia E, Di Gennaro L, Basso M, Giusti B, Papi M, Perini G, Castaman G, De Cristofaro R. Blood Adv 4 3405-3415 (2020)
  34. Structure of von Willebrand factor A1 on polystyrene determined from experimental and calculated sum frequency generation spectra. Roeters SJ, Tronic EH, Baio JE, Castner DG, Weidner T. Biointerphases 13 06E411 (2018)
  35. Von Willebrand factor A1 domain stability and affinity for GPIbα are differentially regulated by its O-glycosylated N- and C-linker. Bonazza K, Iacob RE, Hudson NE, Li J, Lu C, Engen JR, Springer TA. Elife 11 e75760 (2022)
  36. The p.P1127S pathogenic variant lowers von Willebrand factor levels through higher affinity for the macrophagic scavenger receptor LRP1: Clinical phenotype and pathogenic mechanisms. Sacco M, Lancellotti S, Branchini A, Tardugno M, Testa MF, Lunghi B, Bernardi F, Pinotti M, Giusti B, Castaman G, De Cristofaro R. J Thromb Haemost 20 1818-1829 (2022)
  37. A p.Arg127Gln variant in GPIbα LRR5 allosterically enhances affinity for VWF: a novel form of platelet-type VWD. Bury L, Falcinelli E, Kuchi Bhotla H, Mezzasoma AM, Guglielmini G, Tischer A, Moon-Tasson L, Auton M, Gresele P. Blood Adv 6 2236-2246 (2022)
  38. Antithrombotic effect of SP-8008, a benzoic acid derivative, through the selective inhibition of shear stress-induced platelet aggregation. Ngo T, Kim K, Bian Y, Nam G, Park HJ, Lee K, Cho GS, Ryu JM, Lim KM, Chung JH. Br J Pharmacol 177 929-944 (2020)
  39. Oxidation shuts down an auto-inhibitory mechanism of von Willebrand factor. Tsai R, Interlandi G. Proteins 89 731-741 (2021)
  40. Genetic Alterations, DNA Methylation, Alloantibodies and Phenotypic Heterogeneity in Type III von Willebrand Disease. Naveed MA, Abid A, Ali N, Hassan Y, Amar A, Javed A, Qamar K, Mustafa G, Raza A, Saleem U, Hussain S, Shakoor M, Khaliq S, Mohsin S. Genes (Basel) 13 971 (2022)


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  1. The ubiquitin-proteasome proteolytic pathway: destruction for the sake of construction. Glickman MH, Ciechanover A. Physiol Rev 82 373-428 (2002)
  2. Distribution and evolution of von Willebrand/integrin A domains: widely dispersed domains with roles in cell adhesion and elsewhere. Whittaker CA, Hynes RO. Mol Biol Cell 13 3369-3387 (2002)
  3. Conformational regulation of integrin structure and function. Shimaoka M, Takagi J, Springer TA. Annu Rev Biophys Biomol Struct 31 485-516 (2002)
  4. Integrin activation and structural rearrangement. Takagi J, Springer TA. Immunol Rev 186 141-163 (2002)
  5. von Willebrand factor, Jedi knight of the bloodstream. Springer TA. Blood 124 1412-1425 (2014)
  6. Domain structure and organisation in extracellular matrix proteins. Hohenester E, Engel J. Matrix Biol 21 115-128 (2002)
  7. New concepts in von Willebrand disease. Sadler JE. Annu Rev Med 56 173-191 (2005)
  8. Plasmodium sporozoite molecular cell biology. Kappe SH, Buscaglia CA, Nussenzweig V. Annu Rev Cell Dev Biol 20 29-59 (2004)
  9. Cleavage of ultra-large von Willebrand factor by ADAMTS-13 under flow conditions. Dong JF. J Thromb Haemost 3 1710-1716 (2005)
  10. Function of von Willebrand factor in haemostasis and thrombosis. Reininger AJ. Haemophilia 14 Suppl 5 11-26 (2008)
  11. von Willebrand factor to the rescue. De Meyer SF, Deckmyn H, Vanhoorelbeke K. Blood 113 5049-5057 (2009)
  12. Molecular basis of inherited diseases: a structural perspective. Steward RE, MacArthur MW, Laskowski RA, Thornton JM. Trends Genet 19 505-513 (2003)
  13. The matrilins: modulators of extracellular matrix assembly. Klatt AR, Becker AK, Neacsu CD, Paulsson M, Wagener R. Int J Biochem Cell Biol 43 320-330 (2011)
  14. Structural insights into the interactions between platelet receptors and fibrillar collagen. Herr AB, Farndale RW. J Biol Chem 284 19781-19785 (2009)
  15. Complement and the multifaceted functions of VWA and integrin I domains. Springer TA. Structure 14 1611-1616 (2006)
  16. A biophysical view on von Willebrand factor activation. Löf A, Müller JP, Brehm MA. J Cell Physiol 233 799-810 (2018)
  17. Biology and structure of leukocyte β 2 integrins and their role in inflammation. Arnaout MA. F1000Res 5 F1000 Faculty Rev-2433 (2016)
  18. Structure and function of von Willebrand factor. Hassan MI, Saxena A, Ahmad F. Blood Coagul Fibrinolysis 23 11-22 (2012)
  19. Weibel-Palade bodies: a window to von Willebrand disease. Valentijn KM, Eikenboom J. J Thromb Haemost 11 581-592 (2013)
  20. Cleavage of von Willebrand factor by ADAMTS-13 on endothelial cells. López JA, Dong JF. Semin Hematol 41 15-23 (2004)
  21. Synthetic Peptide templates for molecular recognition: recent advances and applications. Singh Y, Dolphin GT, Razkin J, Dumy P. Chembiochem 7 1298-1314 (2006)
  22. Structure and function of snake venom toxins interacting with human von Willebrand factor. Matsui T, Hamako J. Toxicon 45 1075-1087 (2005)
  23. Collagen Binding Proteins of Gram-Positive Pathogens. Arora S, Gordon J, Hook M. Front Microbiol 12 628798 (2021)
  24. Occlusive thrombosis in arteries. Kim D, Bresette C, Liu Z, Ku DN. APL Bioeng 3 041502 (2019)
  25. Functional property of von Willebrand factor under flowing blood. Sugimoto M, Miyata S. Int J Hematol 75 19-24 (2002)
  26. Structure-function relationships of the luteinizing hormone receptor. Puett D, Li Y, Angelova K, Demars G, Meehan TP, Fanelli F, Narayan P. Ann N Y Acad Sci 1061 41-54 (2005)
  27. Inhibition of von Willebrand factor-GPIb/IX/V interactions as a strategy to prevent arterial thrombosis. Bonnefoy A, Vermylen J, Hoylaerts MF. Expert Rev Cardiovasc Ther 1 257-269 (2003)

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  1. Structural basis of collagen recognition by integrin alpha2beta1. Emsley J, Knight CG, Farndale RW, Barnes MJ, Liddington RC. Cell 101 47-56 (2000)
  2. Platelet glycoprotein ibalpha is a counterreceptor for the leukocyte integrin Mac-1 (CD11b/CD18). Simon DI, Chen Z, Xu H, Li CQ, Dong Jf, McIntire LV, Ballantyne CM, Zhang L, Furman MI, Berndt MC, López JA. J Exp Med 192 193-204 (2000)
  3. Subunit interaction maps for the regulatory particle of the 26S proteasome and the COP9 signalosome. Fu H, Reis N, Lee Y, Glickman MH, Vierstra RD. EMBO J 20 7096-7107 (2001)
  4. Role of sialic acid for platelet life span: exposure of beta-galactose results in the rapid clearance of platelets from the circulation by asialoglycoprotein receptor-expressing liver macrophages and hepatocytes. Sørensen AL, Rumjantseva V, Nayeb-Hashemi S, Clausen H, Hartwig JH, Wandall HH, Hoffmeister KM. Blood 114 1645-1654 (2009)
  5. Crystal structure of the wild-type von Willebrand factor A1-glycoprotein Ibalpha complex reveals conformation differences with a complex bearing von Willebrand disease mutations. Dumas JJ, Kumar R, McDonagh T, Sullivan F, Stahl ML, Somers WS, Mosyak L. J Biol Chem 279 23327-23334 (2004)
  6. NMR and mutagenesis evidence for an I domain allosteric site that regulates lymphocyte function-associated antigen 1 ligand binding. Huth JR, Olejniczak ET, Mendoza R, Liang H, Harris EA, Lupher ML, Wilson AE, Fesik SW, Staunton DE. Proc Natl Acad Sci U S A 97 5231-5236 (2000)
  7. Inhibition of von Willebrand factor-mediated platelet activation and thrombosis by the anti-von Willebrand factor A1-domain aptamer ARC1779. Diener JL, Daniel Lagassé HA, Duerschmied D, Merhi Y, Tanguay JF, Hutabarat R, Gilbert J, Wagner DD, Schaub R. J Thromb Haemost 7 1155-1162 (2009)
  8. Conformational changes in tertiary structure near the ligand binding site of an integrin I domain. Oxvig C, Lu C, Springer TA. Proc Natl Acad Sci U S A 96 2215-2220 (1999)
  9. Staphylococcus aureus protein A binding to von Willebrand factor A1 domain is mediated by conserved IgG binding regions. O'Seaghdha M, van Schooten CJ, Kerrigan SW, Emsley J, Silverman GJ, Cox D, Lenting PJ, Foster TJ. FEBS J 273 4831-4841 (2006)
  10. A pH-regulated dimeric bouquet in the structure of von Willebrand factor. Zhou YF, Eng ET, Nishida N, Lu C, Walz T, Springer TA. EMBO J 30 4098-4111 (2011)
  11. The N-terminal flanking region of the A1 domain regulates the force-dependent binding of von Willebrand factor to platelet glycoprotein Ibα. Ju L, Dong JF, Cruz MA, Zhu C. J Biol Chem 288 32289-32301 (2013)
  12. Shear-induced unfolding activates von Willebrand factor A2 domain for proteolysis. Baldauf C, Schneppenheim R, Stacklies W, Obser T, Pieconka A, Schneppenheim S, Budde U, Zhou J, Gräter F. J Thromb Haemost 7 2096-2105 (2009)
  13. A bacterial collagen-binding domain with novel calcium-binding motif controls domain orientation. Wilson JJ, Matsushita O, Okabe A, Sakon J. EMBO J 22 1743-1752 (2003)
  14. Trench-shaped binding sites promote multiple classes of interactions between collagen and the adherence receptors, alpha(1)beta(1) integrin and Staphylococcus aureus cna MSCRAMM. Rich RL, Deivanayagam CC, Owens RT, Carson M, Höök A, Moore D, Symersky J, Yang VW, Narayana SV, Höök M. J Biol Chem 274 24906-24913 (1999)
  15. The snake venom protein botrocetin acts as a biological brace to promote dysfunctional platelet aggregation. Fukuda K, Doggett T, Laurenzi IJ, Liddington RC, Diacovo TG. Nat Struct Mol Biol 12 152-159 (2005)
  16. Calcium modulates force sensing by the von Willebrand factor A2 domain. Jakobi AJ, Mashaghi A, Tans SJ, Huizinga EG. Nat Commun 2 385 (2011)
  17. Structural and functional studies with antibodies to the integrin beta 2 subunit. A model for the I-like domain. Huang C, Zang Q, Takagi J, Springer TA. J Biol Chem 275 21514-21524 (2000)
  18. Cryoelectron microscopy imaging of recombinant and tissue derived vaults: localization of the MVP N termini and VPARP. Mikyas Y, Makabi M, Raval-Fernandes S, Harrington L, Kickhoefer VA, Rome LH, Stewart PL. J Mol Biol 344 91-105 (2004)
  19. Comparative genome analysis of the pathogenic spirochetes Borrelia burgdorferi and Treponema pallidum. Subramanian G, Koonin EV, Aravind L. Infect Immun 68 1633-1648 (2000)
  20. A structural explanation for the antithrombotic activity of ARC1172, a DNA aptamer that binds von Willebrand factor domain A1. Huang RH, Fremont DH, Diener JL, Schaub RG, Sadler JE. Structure 17 1476-1484 (2009)
  21. Structural basis of von Willebrand factor activation by the snake toxin botrocetin. Fukuda K, Doggett TA, Bankston LA, Cruz MA, Diacovo TG, Liddington RC. Structure 10 943-950 (2002)
  22. A novel DFNA9 mutation in the vWFA2 domain of COCH alters a conserved cysteine residue and intrachain disulfide bond formation resulting in progressive hearing loss and site-specific vestibular and central oculomotor dysfunction. Street VA, Kallman JC, Robertson NG, Kuo SF, Morton CC, Phillips JO. Am J Med Genet A 139A 86-95 (2005)
  23. Shear stress-induced unfolding of VWF accelerates oxidation of key methionine residues in the A1A2A3 region. Fu X, Chen J, Gallagher R, Zheng Y, Chung DW, López JA. Blood 118 5283-5291 (2011)
  24. Force-sensitive autoinhibition of the von Willebrand factor is mediated by interdomain interactions. Aponte-Santamaría C, Huck V, Posch S, Bronowska AK, Grässle S, Brehm MA, Obser T, Schneppenheim R, Hinterdorfer P, Schneider SW, Baldauf C, Gräter F. Biophys J 108 2312-2321 (2015)
  25. Identification of heparin as a ligand for the A-domain of Plasmodium falciparum thrombospondin-related adhesion protein. McCormick CJ, Tuckwell DS, Crisanti A, Humphries MJ, Hollingdale MR. Mol Biochem Parasitol 100 111-124 (1999)
  26. Function of the cysteine-rich domain of the haemorrhagic metalloproteinase atrolysin A: targeting adhesion proteins collagen I and von Willebrand factor. Serrano SM, Jia LG, Wang D, Shannon JD, Fox JW. Biochem J 391 69-76 (2005)
  27. Localization of von willebrand factor-binding sites for platelet glycoprotein Ib and botrocetin by charged-to-alanine scanning mutagenesis. Matsushita T, Meyer D, Sadler JE. J Biol Chem 275 11044-11049 (2000)
  28. A new look at Weibel-Palade body structure in endothelial cells using electron tomography. Valentijn KM, Valentijn JA, Jansen KA, Koster AJ. J Struct Biol 161 447-458 (2008)
  29. A new FACIT of the collagen family: COL21A1. Fitzgerald J, Bateman JF. FEBS Lett 505 275-280 (2001)
  30. A novel calcium-binding site of von Willebrand factor A2 domain regulates its cleavage by ADAMTS13. Zhou M, Dong X, Baldauf C, Chen H, Zhou Y, Springer TA, Luo X, Zhong C, Gräter F, Ding J. Blood 117 4623-4631 (2011)
  31. O-linked glycosylation and functional incompatibility of porcine von Willebrand factor for human platelet GPIb receptors. Schulte Am Esch J, Robson SC, Knoefel WT, Hosch SB, Rogiers X. Xenotransplantation 12 30-37 (2005)
  32. The interaction of von Willebrand factor-A1 domain with collagen: mutation G1324S (type 2M von Willebrand disease) impairs the conformational change in A1 domain induced by collagen. Morales LD, Martin C, Cruz MA. J Thromb Haemost 4 417-425 (2006)
  33. Von Willebrand factor-A1 domain binds platelet glycoprotein Ibα in multiple states with distinctive force-dependent dissociation kinetics. Ju L, Chen Y, Zhou F, Lu H, Cruz MA, Zhu C. Thromb Res 136 606-612 (2015)
  34. Mutation distribution in the von Willebrand factor gene related to the different von Willebrand disease (VWD) types in a cohort of VWD patients. Yadegari H, Driesen J, Pavlova A, Biswas A, Hertfelder HJ, Oldenburg J. Thromb Haemost 108 662-671 (2012)
  35. Structural and functional dissection of the adhesive domains of Plasmodium falciparum thrombospondin-related anonymous protein (TRAP). Akhouri RR, Bhattacharyya A, Pattnaik P, Malhotra P, Sharma A. Biochem J 379 815-822 (2004)
  36. A discontinuous autoinhibitory module masks the A1 domain of von Willebrand factor. Deng W, Wang Y, Druzak SA, Healey JF, Syed AK, Lollar P, Li R. J Thromb Haemost 15 1867-1877 (2017)
  37. Changes in thermodynamic stability of von Willebrand factor differentially affect the force-dependent binding to platelet GPIbalpha. Auton M, Sedlák E, Marek J, Wu T, Zhu C, Cruz MA. Biophys J 97 618-627 (2009)
  38. The structure of Mycobacterium tuberculosis MPT51 (FbpC1) defines a new family of non-catalytic alpha/beta hydrolases. Wilson RA, Maughan WN, Kremer L, Besra GS, Fütterer K. J Mol Biol 335 519-530 (2004)
  39. Dynamic force spectroscopy of glycoprotein Ib-IX and von Willebrand factor. Arya M, Kolomeisky AB, Romo GM, Cruz MA, López JA, Anvari B. Biophys J 88 4391-4401 (2005)
  40. Identification of the C3b binding site in a recombinant vWF-A domain of complement factor B by surface-enhanced laser desorption-ionisation affinity mass spectrometry and homology modelling: implications for the activity of factor B. Hinshelwood J, Spencer DI, Edwards YJ, Perkins SJ. J Mol Biol 294 587-599 (1999)
  41. Misfolding of vWF to pathologically disordered conformations impacts the severity of von Willebrand disease. Tischer A, Madde P, Moon-Tasson L, Auton M. Biophys J 107 1185-1195 (2014)
  42. The two variants of the Streptococcus pneumoniae pilus 1 RrgA adhesin retain the same function and elicit cross-protection in vivo. Moschioni M, Emolo C, Biagini M, Maccari S, Pansegrau W, Donati C, Hilleringmann M, Ferlenghi I, Ruggiero P, Sinisi A, Pizza M, Norais N, Barocchi MA, Masignani V. Infect Immun 78 5033-5042 (2010)
  43. Crystal structure of EMS16 in complex with the integrin alpha2-I domain. Horii K, Okuda D, Morita T, Mizuno H. J Mol Biol 341 519-527 (2004)
  44. The Membrane-associated form of the DNA repair protein Ku is involved in cell adhesion to fibronectin. Monferran S, Muller C, Mourey L, Frit P, Salles B. J Mol Biol 337 503-511 (2004)
  45. The linker between the D3 and A1 domains of vWF suppresses A1-GPIbα catch bonds by site-specific binding to the A1 domain. Tischer A, Cruz MA, Auton M. Protein Sci 22 1049-1059 (2013)
  46. GPIbα-vWF rolling under shear stress shows differences between type 2B and 2M von Willebrand disease. Coburn LA, Damaraju VS, Dozic S, Eskin SG, Cruz MA, McIntire LV. Biophys J 100 304-312 (2011)
  47. Glycoprotein Ibalpha inhibitor complex structure reveals a combined steric and allosteric mechanism of von Willebrand factor antagonism. McEwan PA, Andrews RK, Emsley J. Blood 114 4883-4885 (2009)
  48. Glycoprotein IIb-IIIa-dependent aggregation by glycoprotein Ibalpha is reinforced by a Src family kinase inhibitor (PP1)-sensitive signalling pathway. Marshall SJ, Asazuma N, Best D, Wonerow P, Salmon G, Andrews RK, Watson SP. Biochem J 361 297-305 (2002)
  49. Impact of O-linked glycosylation of the VWF-A1-domain flanking regions on platelet interaction. Schulte am Esch J, Robson SC, Knoefel WT, Eisenberger CF, Peiper M, Rogiers X. Br J Haematol 128 82-90 (2005)
  50. Mapping von Willebrand factor A domain binding sites on a snake venom metalloproteinase cysteine-rich domain. Pinto AF, Terra RM, Guimaraes JA, Fox JW. Arch Biochem Biophys 457 41-46 (2007)
  51. Structure of the leech protein saratin and characterization of its binding to collagen. Gronwald W, Bomke J, Maurer T, Domogalla B, Huber F, Schumann F, Kremer W, Fink F, Rysiok T, Frech M, Kalbitzer HR. J Mol Biol 381 913-927 (2008)
  52. WARP is a new member of the von Willebrand factor A-domain superfamily of extracellular matrix proteins. Fitzgerald J, Tay Ting S, Bateman JF. FEBS Lett 517 61-66 (2002)
  53. A Bethlem myopathy Gly to Glu mutation in the von Willebrand factor A domain N2 of the collagen alpha3(VI) chain interferes with protein folding. Sasaki T, Hohenester E, Zhang RZ, Gotta S, Speer MC, Tandan R, Timpl R, Chu ML. FASEB J 14 761-768 (2000)
  54. Sulfatides inhibit platelet adhesion to von Willebrand factor in flowing blood. Borthakur G, Cruz MA, Dong JF, McIntire L, Li F, López JA, Thiagarajan P. J Thromb Haemost 1 1288-1295 (2003)
  55. BB0172, a Borrelia burgdorferi outer membrane protein that binds integrin α3β1. Wood E, Tamborero S, Mingarro I, Esteve-Gassent MD. J Bacteriol 195 3320-3330 (2013)
  56. Force-induced on-rate switching and modulation by mutations in gain-of-function von Willebrand diseases. Kim J, Hudson NE, Springer TA. Proc Natl Acad Sci U S A 112 4648-4653 (2015)
  57. Antigenic complementarity in the origins of autoimmunity: a general theory illustrated with a case study of idiopathic thrombocytopenia purpura. Root-Bernstein R, Couturier J. Clin Dev Immunol 13 49-65 (2006)
  58. Recessive COL6A2 C-globular missense mutations in Ullrich congenital muscular dystrophy: role of the C2a splice variant. Zhang RZ, Zou Y, Pan TC, Markova D, Fertala A, Hu Y, Squarzoni S, Reed UC, Marie SKN, Bönnemann CG, Chu ML. J Biol Chem 285 10005-10015 (2010)
  59. Specific electrostatic interactions between charged amino acid residues regulate binding of von Willebrand factor to blood platelets. Interlandi G, Yakovenko O, Tu AY, Harris J, Le J, Chen J, López JA, Thomas WE. J Biol Chem 292 18608-18617 (2017)
  60. Structural characterisation of sporozoite components for a multistage, multi-epitope, anti-malarial vaccine. Patarroyo ME, Cifuentes G, Rodríguez R. Int J Biochem Cell Biol 40 543-557 (2008)
  61. The second von Willebrand type A domain of cochlin has high affinity for type I, type II and type IV collagens. Nagy I, Trexler M, Patthy L. FEBS Lett 582 4003-4007 (2008)
  62. Mutational Constraints on Local Unfolding Inhibit the Rheological Adaptation of von Willebrand Factor. Tischer A, Campbell JC, Machha VR, Moon-Tasson L, Benson LM, Sankaran B, Kim C, Auton M. J Biol Chem 291 3848-3859 (2016)
  63. vWA proteins of Leptospira interrogans induce hemorrhage in leptospirosis by competitive inhibition of vWF/GPIb-mediated platelet aggregation. Fang JQ, Imran M, Hu WL, Ojcius DM, Li Y, Ge YM, Li KX, Lin X, Yan J. EBioMedicine 37 428-441 (2018)
  64. Structure and dynamics of the platelet integrin-binding C4 domain of von Willebrand factor. Xu ER, von Bülow S, Chen PC, Lenting PJ, Kolšek K, Aponte-Santamaría C, Simon B, Foot J, Obser T, Schneppenheim R, Gräter F, Denis CV, Wilmanns M, Hennig J. Blood 133 366-376 (2019)
  65. Conformation of the von Willebrand factor/factor VIII complex in quasi-static flow. Parker ET, Lollar P. J Biol Chem 296 100420 (2021)
  66. Structure of Plasmodium falciparum TRAP (thrombospondin-related anonymous protein) A domain highlights distinct features in apicomplexan von Willebrand factor A homologues. Pihlajamaa T, Kajander T, Knuuti J, Horkka K, Sharma A, Permi P. Biochem J 450 469-476 (2013)
  67. Identification and characterization of a novel mutation in von Willebrand factor causing type 2B von Willebrand's disease. Facey DA, Favaloro EJ, Koutts J, Berndt MC, Hertzberg MS. Br J Haematol 105 538-541 (1999)
  68. Metal-dependent conformational changes in a recombinant vWF-A domain from human factor B: a solution study by circular dichroism, fourier transform infrared and (1)H NMR spectroscopy. Hinshelwood J, Perkins SJ. J Mol Biol 298 135-147 (2000)
  69. A template-assembled synthetic protein surface mimetic of the von Willebrand factor A1 domain inhibits botrocetin-induced platelet aggregation. Hauert J, Fernandez-Carneado J, Michielin O, Mathieu S, Grell D, Schapira M, Spertini O, Mutter M, Tuchscherer G, Kovacsovics T. Chembiochem 5 856-864 (2004)
  70. Clinical and laboratory phenotype variability in type 2M von Willebrand disease. Doruelo AL, Haberichter SL, Christopherson PA, Boggio LN, Gupta S, Lentz SR, Shapiro AD, Montgomery RR, Flood VH. J Thromb Haemost 15 1559-1566 (2017)
  71. Evidence from limited proteolysis of a ristocetin-induced conformational change in human von Willebrand factor that promotes its binding to platelet glycoprotein Ib-IX-V. Kang M, Wilson L, Kermode JC. Blood Cells Mol Dis 40 433-443 (2008)
  72. Staphylococcus aureus vWF-binding protein triggers a strong interaction between clumping factor A and host vWF. Viljoen A, Viela F, Mathelié-Guinlet M, Missiakas D, Pietrocola G, Speziale P, Dufrêne YF. Commun Biol 4 453 (2021)
  73. A monoclonal antibody directed against human von Willebrand factor induces type 2B-like alterations. Ulrichts H, Harsfalvi J, Bene L, Matko J, Vermylen J, Ajzenberg N, Baruch D, Deckmyn H, Tornai I. J Thromb Haemost 2 1622-1628 (2004)
  74. Identification of new type 2B von Willebrand disease mutations: Arg543Gln, Arg545Pro and Arg578Leu. Hilbert L, Gaucher C, Abgrall JF, Parquet A, Trzeciak C, Mazurier C. Br J Haematol 103 877-884 (1998)
  75. Storage and secretion of naturally occurring von Willebrand factor A domain variants. Groeneveld DJ, Wang JW, Mourik MJ, Dirven RJ, Valentijn KM, Voorberg J, Reitsma PH, Eikenboom J. Br J Haematol 167 529-540 (2014)
  76. Surface grafting onto template-assembled synthetic protein scaffolds in molecular recognition. Fernandez-Carneado J, Grell D, Durieux P, Hauert J, Kovacsovics T, Tuchscherer G. Biopolymers 55 451-458 (2000)
  77. The functions of the A1A2A3 domains in von Willebrand factor include multimerin 1 binding. Parker DN, Tasneem S, Farndale RW, Bihan D, Sadler JE, Sebastian S, de Groot PG, Hayward CP. Thromb Haemost 116 87-95 (2016)
  78. A new mutation, S1285F, within the A1 loop of von Willebrand factor induces a conformational change in A1 loop with abnormal binding to platelet GPIb and botrocetin causing type 2M von Willebrand disease. Stepanian A, Ribba AS, Lavergne JM, Fressinaud E, Juhan-Vague I, Mazurier C, Girma JP, Meyer D. Br J Haematol 120 643-651 (2003)
  79. Glycoprotein Ibalpha can mediate endothelial cell migration on von Willebrand factor-containing substrata. Lian J, Guoping C, Shapiro SS, Tran LP, Beacham DA. Exp Cell Res 252 114-122 (1999)
  80. Staphylococcus aureus iron-regulated surface determinant B (IsdB) protein interacts with von Willebrand factor and promotes adherence to endothelial cells. Alfeo MJ, Pagotto A, Barbieri G, Foster TJ, Vanhoorelbeke K, De Filippis V, Speziale P, Pietrocola G. Sci Rep 11 22799 (2021)
  81. The In Vitro Replication Cycle of Achromobacter xylosoxidans and Identification of Virulence Genes Associated with Cytotoxicity in Macrophages. Pickrum AM, Riegert MO, Wells C, Brockman K, Frank DW. Microbiol Spectr 10 e0208322 (2022)
  82. Data on the purification and crystallization of the loss-of-function von Willebrand disease variant (p.Gly1324Ser) of the von Willebrand factor A1 domain. Campbell JC, Tischer A, Machha V, Moon-Tasson L, Sankaran B, Kim C, Auton M. Data Brief 7 1700-1706 (2016)
  83. Differential surface activation of the A1 domain of von Willebrand factor. Tronic EH, Yakovenko O, Weidner T, Baio JE, Penkala R, Castner DG, Thomas WE. Biointerphases 11 029803 (2016)
  84. Structural diversity of a collagen-binding matrix protein from the byssus of blue mussels upon refolding. Suhre MH, Scheibel T. J Struct Biol 186 75-85 (2014)
  85. Type 2B von Willebrand's disease in thirteen individuals from five unrelated Australian families: phenotype and genotype correlations. Facey DA, Favaloro EJ, Maxwell E, Baker R, Hertzberg MS. Am J Hematol 63 197-199 (2000)
  86. Helical self-assembly of a mucin segment suggests an evolutionary origin for von Willebrand factor tubules. Javitt G, Fass D. Proc Natl Acad Sci U S A 119 e2116790119 (2022)
  87. "Bleeding in the jungle". Arbelaez A, Niemann J, Freney R, Othman M, Emsley J, Mohammed S, Favaloro EJ. Am J Hematol 90 843-846 (2015)
  88. Ca2+-based allosteric switches and shape shifting in RGLG1 VWA domain. Wang Q, Chen Y, Li S, Yang W, Sun L, Jang M, Wu X, Wang Q, Chen L, Wu Y. Comput Struct Biotechnol J 18 821-833 (2020)
  89. Functional display of platelet-binding VWF fragments on filamentous bacteriophage. Yee A, Tan FL, Ginsburg D. PLoS One 8 e73518 (2013)
  90. Feature architecture aware phylogenetic profiling indicates a functional diversification of type IVa pili in the nosocomial pathogen Acinetobacter baumannii. Iruegas R, Pfefferle K, Göttig S, Averhoff B, Ebersberger I. PLoS Genet 19 e1010646 (2023)
  91. Identification of amino acid residues responsible for von Willebrand factor binding to sulfatide by charged-to-alanine-scanning mutagenesis. Nakayama T, Matsushita T, Yamamoto K, Mutsuga N, Kojima T, Katsumi A, Nakao N, Sadler JE, Naoe T, Saito H. Int J Hematol 87 363-370 (2008)
  92. Subunit Flexibility of Multimeric von Willebrand Factor/Factor VIII Complexes. Parker ET, Haberichter SL, Lollar P. ACS Omega 7 31183-31196 (2022)
  93. A first Taiwanese Chinese family of type 2B von Willebrand disease with R1306W mutation. Shen MC, Lin JS, Lin DS, Hsu SC, Lin B. Thromb Res 112 291-295 (2003)
  94. Comment A hitchhiker's guide to the galaxy--an H. pylori travel guide. Handin RI. Gastroenterology 124 1983-1985 (2003)
  95. CD11b of Ovis canadensis and Ovis aries: molecular cloning and characterization. Lawrence PK, Srikumaran S. Vet Immunol Immunopathol 119 287-298 (2007)
  96. Conformational activation and inhibition of von Willebrand factor by targeting its autoinhibitory module. Arce NA, Markham-Lee Z, Liang Q, Najmudin S, Legan ER, Dean G, Su AJ, Wilson MS, Sidonio RF, Lollar P, Emsley J, Li R. Blood 143 1992-2004 (2024)
  97. Generation and characterization of a recombinant single chain Fv antibody to von Willebrand factor A1 domain from phage display library. Zhu H, Wang Y, Jiang M, Ji S, Bai X, Ruan C. Thromb Res 116 385-391 (2005)
  98. N1421K mutation in the glycoprotein Ib binding domain impairs ristocetin- and botrocetin-mediated binding of von Willebrand factor to platelets. Lanke E, Kristoffersson AC, Isaksson C, Holmberg L, Lethagen S. Eur J Haematol 81 384-390 (2008)
  99. Vwf K1362A resulted in failure of protein synthesis in mice. Sanda N, Suzuki N, Suzuki A, Kanematsu T, Kishimoto M, Hasuwa H, Takagi A, Kojima T, Matsushita T, Nakamura S. Int J Hematol 107 428-435 (2018)
  100. Cryptic Extensibility in von Willebrand Factor Revealed by Molecular Nanodissection. Csányi MC, Sziklai D, Feller T, Hársfalvi J, Kellermayer M. Int J Mol Sci 25 7296 (2024)
  101. Physical Characteristics of von Willebrand Factor Binding with Platelet Glycoprotein Ibɑ Mutants at Residue 233 Causing Various Biological Functions. Nakayama M, Goto S, Goto S. TH Open 6 e421-e428 (2022)
  102. Platelet aggregation by membrane-expressed A1 domains of von Willebrand Factor is dependent on residues Asp 560 and Gly 561. Schulte am Esch J, Robson SC, Cruz MA. Biochem Biophys Res Commun 302 873-877 (2003)


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