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- W2019413657 abstract "Journal of Clinical Laboratory AnalysisVolume 1, Issue 4 p. 353-362 Review Article Classification of von willebrand disease Zaverio M. Ruggeri, Corresponding Author Zaverio M. Ruggeri Division of Experimental Hemostasis, Department of Basic and Clinical Research, Scripps Clinic and Research Foundation, La JoHa, CaliforniaZayerio M. Ruggeri, MD (BCR8), Scripps Clinic and Research Foundation, 10666 N. Torrey Pines Road, La Jolla. CA92037Search for more papers by this authorTheodore S. Zimmerman, Theodore S. Zimmerman Division of Experimental Hemostasis, Department of Basic and Clinical Research, Scripps Clinic and Research Foundation, La JoHa, CaliforniaSearch for more papers by this author Zaverio M. Ruggeri, Corresponding Author Zaverio M. Ruggeri Division of Experimental Hemostasis, Department of Basic and Clinical Research, Scripps Clinic and Research Foundation, La JoHa, CaliforniaZayerio M. Ruggeri, MD (BCR8), Scripps Clinic and Research Foundation, 10666 N. Torrey Pines Road, La Jolla. CA92037Search for more papers by this authorTheodore S. Zimmerman, Theodore S. Zimmerman Division of Experimental Hemostasis, Department of Basic and Clinical Research, Scripps Clinic and Research Foundation, La JoHa, CaliforniaSearch for more papers by this author First published: 1987 https://doi.org/10.1002/jcla.1860010408Citations: 7AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat References 1 von Willebrand EA: Hereditär pseudohemofili. Finska Lakaresallskapets Handlingar, 67: 7–112, 1926. 2 Zimmerman TS, Ratnoff, OD, Powell AE: Immunologie differentiation of classic hemophilia (Factor VIII deficiency) and von Wille-brand's disease. With observations on combined deficiencies of antihemophilic factor and proaccelerin (Factor V) and on an acquired circulating anticoagulant against antihemophilic factor. J Clin Invest 40: 244–254, 1971. 3 Jaffe EA, Hoyer LW, Nachman RL: Synthesis of von Willebrand factor by cultured human endothelial cells. Proc Natl Acad Sci USA 71: 1906 1909, 1974. 4 Sporn LA, Chavin SI, Marder VJ, Wagner DD: Biosynthesis of von Willebrand protein by human megakaryocytes. J Clin Invest 76: 1102–1106, 1985. 5 Ginsburg D, Handin RI, Bonthron DT, Donion TA, Bruns GA, Latt SA, Orkin SH: Human von Willebrand factor (vWF): Isolation of complementary DNA (cDNA) clones and chromosomal localization. Science 228: 1401–1406, 1985. 6 Verweij CL, de Vries CJM, Distel B, van Zonneveld A-J, von Kessel AG, Van Mourik JA, Pannekoek H: Construction of cDNA coding for human von Willebrand factor using antibody probes for colony-screening and mapping of the chromosomal gene. Nucleic Acids Res 13: 4699–4717, 1985. 7 Bonthron D, Orr EC, Mitsock LM, Ginsburg D, Handin RI, Orkin SH: Nucleotide sequence of pre-pro-von Willebrand factor cDNA. Nucleic Acids Res 14: 7125–7127, 1986. 8 Fowler WE, Fretto LJ, Hamilton KK, Erickson HP, McKee PA: Substructure of human von Willebrand factor. J Clin Invest 76: 1491–1500, 1985. 9 Wagner DD, Marder VJ: Biosynthesis of von Willebrand protein by human endothelial cells: Processing steps and their intracellular localization. J Cell Biol 99: 2123–2130, 1984. 10 Fay PJ, Kawai Y, Wagner DD, Ginsburg D, Bonthron D, Ohlsson- Wilhelm BM, Chavin SI, Abraham GN, Handin RI, Orkin SH, Montgomery RR, Marder VJ: Propolypeptide of von Willebrand factor circulates in blood and is identical to von Willebrand antigen II. Science 232: 995–998, 1986. 11 McCarroll DR, Levin EG, Montgomery RR: Endothelial cell synthesis of von Willebrand antigen II, von Willebrand factor, and von Willebrand factor/von Willebrand antigen II complex. J Clin Invest 75: 1089–1095, 1985. 12 Scott JP, Montgomery RR: Platelet von Willebrand's antigen II: active release by aggregating agents and a marker of platelet release reaction in vivo. Blood 58: 1075–1080, 1981. 13 Montgomery RR, Zimmerman TS: von Willebrand's disease antigen II. A new plasma and platelet antigen deficient in severe von Willebrand's disease. J Clin Invest 61: 1498–1507, 1978. 14 Titani K, Kumar S, Takio K, Ericsson LH, Wade RD, Ashida K, Walsh KA, Chopek MW, Sadler JE, Fujikawa K: Amino acid sequence of human von Willebrand factor. Biochemistry 25: 3171–3184, 1986. 15 Lynch DC, Zimmerman TS, Collins CJ, Brown M, Morin MJ, Ling EH, Livingston DM: Molecular cloning of cDNA for human von Willebrand factor: Authentication by a new method. Cell 41: 49–56, 1985. 16 Sadler JE, Shelton-Inloes BB, Sorace JM, Harlan JM, Titani K, Davie EW: Cloning and characterization of two cDNAs coding for human von Willebrand factor. Proc Natl Acad Sci USA 82: 6394–6398, 1985. 17 Wagner DD, Mayadas T, Marder VJ: Initial glycosylation and acidic pH in the Golgi apparatus are required for multimerization of von Willebrand factor. J Cell Biol 102: 1320–1324, 1986. 18 Lynch DC, Zimmerman TS, Kirby EP, Livingston DM: Subunit composition of oligomeric human von Willebrand factor. J Biol Chem 258: 12757–12760, 1983. 19 Wagner DD, Marder VJ: Biosynthesis of von Willebrand protein by human endothelial cells: Identification of a large precursor polypeptide chain. J Biol Chem 258: 2065–2067, 1983. 20 Montgomery RR, Dent J, Schmidt W, Kyrle P, Niessner H, Ruggeri ZM, Zimmerman TS: Hereditary persistence of circulating pro von Willebrand factor (pro-vWF). Circulation 74: II-406a, 1986. 21 Loesberg C, Gonsalves MD, Zandbergen J, Willems C, Van Aken WG, Stel HV, Van Mourik JA, DeGroot PG: The effect of calcium on the secretion of factor VIII-related antigen by cultured human endothelial cells. Biochim Biophys Acta 763: 160–168, 1983. 22 Sporn LA, Marder VJ, Wagner DD: Inducible secretion of large, biologically potent von Willebrand factor multimers. Cell 46: 185–190, 1986. 23 Sussman II, Rand JH: Subendothelial deposition of von Willebrand's factor requires the presence of endothelial cells. 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J Clin Invest 74: 1730–1741, 1984. 29 Vehar GA, Davie EW: Preparation and properties of bovine factor VIII (antihemophilic factor). Biochemistry 19: 401–410, 1980. 30 Fulcher CA, Zimmerman TS: Characterization of the human factor VIII procoagulant protein with a heterologous precipitating antibody. Proc Natl Acad Sci USA 79: 1648–1652, 1982. 31 Weiss HJ, Sussman II, Hoyer LW: Stabilization of factor VIII in plasma by the von Willebrand factor. Studies on posttransfusion and dissociated factor VIII and in patients with von Willebrand's disease. J Clin Invest 60: 390–404, 1977. 32 Ruggeri ZM, De Marco L, Gatti L. Bader R, Montgomery RR: Platelets have more than one binding site for von Willebrand factor. J Clin Invest 72: 1–12, 1983. 33 Fujimura Y, Titani K, Holland LZ, Russell SR, Roberts JR, Elder JH, Ruggeri ZM, Zimmerman TS: von Willebrand factor. A reduced and alkylated 52/48-kDa fragment beginning at amino acid residue 449 contains the domain interacting with platelet glycoprotein Ib. J Biol Chem 261: 381–385, 1986. 34 Fujimura Y, Titani K, Holland LZ, Roberts JR, Kostel P, Ruggeri ZM, Zimmerman TS: A heparin-binding domain of human von Willebrand factor. Characterization and localization to a tryptic fragment extending from amino acid residue Val-449 to Lys-728. J Biol Chem 262: 1734–1739, 1987. 35 Pareti FI, Fujimura Y, Dent JA, Holland LZ, Zimmerman TS, Ruggeri ZM: Isolation and characterization of a collagen binding domain in human von Willebrand factor. J Biol Chem 261: 15310–15315, 1986. 36 Plow EF, Pierschbacher MD, Ruoslahti E, Marguerie GA, Ginsberg MH: The effect of Arg-Gly-Asp-containing peptides on fibrinogen and von Willebrand factor binding to platelets. Proc Natl Acad Sci USA 82: 8057–8061, 1985. 37 Doolittle RF, Watt KWK, Colltrell BA, Strong DD, Riley M: The amino acid sequence of the α-chain of human fibrinogen. Nature 280: 464–468, 1979. 38 Pierschbacher MD, Ruoslahti E: Variants of the cell recognition site of fibronectin that retain attachment-promoting activity. Proc Natl Acad Sci USA 81: 5985–5988, 1984. 39 Fretto LJ, Fowler WE, McCaslin DR, Erickson HP, McKee PA: Substructure of human von Willebrand factor. Proteolysis by V8 and characterization of two functional domains. J Biol Chem 261: 15679–15689, 1986. 40 Kalafatis M, Takahashi Y, Girma JP, Meyer D: A monomeric von Willebrand factor fragment (Spl) located between amino acid residues 911 and 1365 contains a domain interacting with collagen. Blood 68: 348a, 1986. 41 Roth GJ, Titani K, Hoyer LW, Hickey MJ: Localization of binding sites within human von Willebrand factor for monomeric Type III collagen. Biochemistry 25: 8357–8361, 1986. 42 Foster PA, Fulcher CA, Marti T, Titani K, Zimmerman TS: A major factor VIII binding domain resides within the amino-terminal 272 amino acid residues of von Willebrand factor. J Biol Chem 262: 8443, 1987. 43 De Marco L, Girolami A, Russell S, Ruggeri ZM: Interaction of asialo von Willebrand factor with glycoprotein Ib induces fibrinogen binding to the glycoprotein IIb/IIIa complex and mediates platelet aggregation. J Clin Invest 75: 1198–1203, 1985. 44 De Marco L, Girolami A, Zimmerman TS, Ruggeri ZM: Interaction of purified IIB von Willebrand factor with the platelet membrane glycoprotein Ib induces fibrinogen binding to the glycoprotein IIb/IIIa complex and initiates aggregation. Proc Natl Acad Sci USA 82: 7424–7428, 1985. 45 Gralnick HR, Williams SB, Coller BS: Asialo von Willebrand factor interactions with platelets. Interdependence of glycoproteins Ib and IIb/IIIa for binding and aggregation. 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