The role of the D1 domain of the von Willebrand factor propeptide in multimerization of VWF

Blood

Volumn 100, Issue 5, 2002, Pages 1699-1706

  Rosenberg, Jonathan B ^a,b   Haberichter, Sandra L ^a,b   Jozwiak, Mary A ^a,b   Vokac, Elizabeth A ^a,b   Kroner, Philip A ^a,b   Fahs, Scot A ^a,b   Kawai, Yohko ^a,b   Montgomery, Robert R ^a,b  

^a BLOOD RESEARCH INSTITUTE   (United States)

^b MEDICAL COLLEGE OF WISCONSIN   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CHAPERONE; CYSTEINE; RISTOCETIN; SERINE; TYROSINE; VON WILLEBRAND FACTOR;

ALLELE; AMINO TERMINAL SEQUENCE; ARTICLE; BIOSYNTHESIS; CONTROLLED STUDY; GENE MUTATION; GENETIC ANALYSIS; HUMAN; HUMAN CELL; PRIORITY JOURNAL; PROTEIN BINDING; PROTEIN DOMAIN;

EID: 0036721163     PISSN: 00064971     EISSN: None     Source Type: Journal    
DOI: 10.1182/blood-2002-03-0789     Document Type: Article

References (38)

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2. Full-length von Willebrand factor (vWF) cDNA encodes a highly repetitive protein considerably larger than the mature vWF subunit
3. Intracellular trafficking of factor VIII to von Willebrand factor storage granules
4. Immunolocalization of von Willebrand protein in Weibel-Palade bodies of human endothelial cells
5. Domains involved in multimer assembly of von willebrand factor (vWF): Multimerization is independent of dimerization
6. von Willebrand disease
7. Impact, diagnosis and treatment of von Willebrand disease
8. von Willebrand factor storage and multimerization: 2 Independent intracellular processes
9. Requirement for both D domains of the propolypeptide in von Willebrand factor multimerization and storage
10. Induction of specific storage organelles by von Willebrand factor propolypeptide
11. Identification of disulfide-bridged substructures within human von Willebrand factor
12. Intramolecular chaperone: The role of the pro-peptide in protein folding
13. Intramolecular chaperones: Polypeptide extensions that modulate protein folding
14. Expressed full-length von Willebrand factor containing missense mutations linked to type IIB von Willebrand disease shows enhanced binding to platelets
15. Enzymatic amplification of platelet-specific messenger RNA using the polymerase chain reaction
16. Expressing a human proinsulin cDNA in a mouse ACTH-secreting cell: Intracellular storage, proteolytic processing, and secretion on stimulation
17. Abnormal binding of factor VIII is linked with the substitution of glutamine for arginine 91 in von Willebrand factor in a variant form of von Willebrand disease
18. The defective interaction between von Willebrand factor and factor VIII in a patient with type 1 von Willebrand disease is caused by substitution of Arg19 and His54 in mature von Willebrand factor
19. The rapid differentiation of type IIb von Willebrand's disease from platelet-type (pseudo-) von Willebrand's disease by the "neutral" monoclonal antibody binding assay
20. New variant of von Willebrand disease with defective binding to factor VIII
21. Genetic induction of a releasable pool of factor VIII in human endothelial cells
22. von Willebrand factor proteolytic processing and multimerization precede the formation of Weibel-Palade bodies
23. Persistence of pro-vWf, an inherited cause of reduced factor VIII binding, and its comparison with other factor VIII binding vWf mutants
24. Type 1 von Willebrand disease mutation Cys 1149Arg causes intracellular retention and degradation of heterodimers: A possible general mechanism for dominant mutations of ollgomeric proteins
25. Characterization of von Willebrand factor gene defects in two unrelated patients with type IIC von Willebrand disease
26. Vicinal cysteines in the prosequence play a role in von Willebrand factor multimer assembly
27. The propeptide of von Willebrand factor independently mediates the assembly of von Willebrand multimers
28. In vitro multimerization of von Willebrand factor is triggered by low pH: Importance of the propolypeptide and free sulfhydryls
29. A novel von Willebrand disease-causing mutation (Arg273Trp) in the von Willebrand factor propeptide that results in defective multimerization and secretion
30. A new candidate mutation (N528S) within the von Willebrand factor propeptide identified in a Japanese patient with phenotype IIC of von Willebrand disease
31. Identification of a candidate missense mutation in a family with von Willebrand disease type IIC
32. Folding pathway mediated by an intramolecular chaperone
33. The propeptide of preprosomatostatin mediates intracellular transport and secretion of alpha-globin from mammalian cells
34. The pro region of human intestinal lactase-phlorizin hydrolase
35. Dual function of the propeptide of prouroguanylin in the folding of the mature peptide: Disulfide-coupled folding and dimerization
36. Comparison of the 5′-flanking sequences of the human and bovine von Willebrand factor-encoding genes reveals alternation of highly homologous domains with species-specific Alu-type repeats
37. Assembly of multimeric von Willebrand factor directs sorting of P-selectin
38. Biogenesis of von Willebrand factor-containing organelles in heterologous transfected CV-1 cells