Variant von Willebrand's disease: characterization of two subtypes by analysis of multimeric composition of factor VIII/von Willebrand factor in plasma and platelets.
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Initial glycosylation and acidic pH in the Golgi apparatus are required for multimerization of von Willebrand factorImmunolocalization of von Willebrand protein in Weibel-Palade bodies of human endothelial cellsMolecular basis of von Willebrand disease type IIB. Candidate mutations cluster in one disulfide loop between proposed platelet glycoprotein Ib binding sequencesUse of ristocetin cofactor activity in the management of von Willebrand disease.Control of von Willebrand factor multimer size by thrombospondin-1.Plasma of patients with Upshaw-Schulman syndrome, a congenital deficiency of von Willebrand factor-cleaving protease activity, enhances the aggregation of normal platelets under high shear stress.Predicting response to plasma exchange in patients with thrombotic thrombocytopenic purpura with measurement of vWF-cleaving protease activity.Sialyltransferase ST3Gal-IV operates as a dominant modifier of hemostasis by concealing asialoglycoprotein receptor ligands.Diagnosis and follow-up of thrombotic thrombocytopenic purpura by means of von Willebrand factor collagen binding assay.Two newborn-onset patients of Upshaw-Schulman syndrome with distinct subsequent clinical courses.Properties of human asialo-factor VIII. A ristocetin-independent platelet-aggregating agent.Von Willebrand's disease and severe gastrointestinal bleeding. Report of a kindred.Identification of a cleavage site directing the immunochemical detection of molecular abnormalities in type IIA von Willebrand factor.Mediation of fibrin-induced release of von Willebrand factor from cultured endothelial cells by the fibrin beta chainvon Willebrand factor synthesized by endothelial cells from a patient with type IIB von Willebrand disease supports platelet adhesion normally but has an increased affinity for platelets.von Willebrand factor mutation enhancing interaction with platelets in patients with normal multimeric structure.Von Willebrand's disease: a clinical and molecular enigma. Twelfth Annual Paul M. Aggeler Memorial Lecture.Quantification of perioperative changes in von Willebrand factor and factor VIII during elective orthopaedic surgery in normal individuals.Distinct abnormalities in the interaction of purified types IIA and IIB von Willebrand factor with the two platelet binding sites, glycoprotein complexes Ib-IX and IIb-IIIaThe molecular biology of von Willebrand disease.Platelet von Willebrand factor in inherited and acquired bleeding disordersSubunit composition of plasma von Willebrand factor. Cleavage is present in normal individuals, increased in IIA and IIB von Willebrand disease, but minimal in variants with aberrant structure of individual oligomers (types IIC, IID, and IIE).Von Willebrand factor and von Willebrand disease.von Willebrand protein facilitates platelet incorporation in polymerizing fibrinFibrin induces release of von Willebrand factor from endothelial cells.Substructure of human von Willebrand factor.Functional domains on von Willebrand factor. Recognition of discrete tryptic fragments by monoclonal antibodies that inhibit interaction of von Willebrand factor with platelets and with collagen.Carbohydrate moiety of von Willebrand factor is not necessary for maintaining multimeric structure and ristocetin cofactor activity but protects from proteolytic degradationGerm-line mosaicism for a valine-to-methionine substitution at residue 553 in the glycoprotein Ib-binding domain of von Willebrand factor, causing type IIB von Willebrand diseaseSuccessful aortic aneurysm repair in a woman with severe von Willebrand (type 3) disease.Type IIB von Willebrand factor with normal sialic acid content induces platelet aggregation in the absence of ristocetin. Role of platelet activation, fibrinogen, and two distinct membrane receptorsTransplantation of normal bone marrow into a pig with severe von Willebrand's disease.Genetic linkage of two intragenic restriction fragment length polymorphisms with von Willebrand's disease type IIA. Evidence for a defect in the von Willebrand factor geneCellular localization of type 1 plasminogen activator inhibitor messenger RNA and protein in murine renal tissuevon Willebrand Factor-Rich Platelet Thrombi in the Liver Cause Sinusoidal Obstruction Syndrome following Oxaliplatin-Based ChemotherapyElectron microscopy of human factor VIII/Von Willebrand glycoprotein: effect of reducing reagents on structure and functionBiosynthesis of von Willebrand protein by human endothelial cells: processing steps and their intracellular localization.Inhibition of disulfide bonding of von Willebrand protein by monensin results in small, functionally defective multimersComposition of the von Willebrand factor storage organelle (Weibel-Palade body) isolated from cultured human umbilical vein endothelial cellsVon Willebrand protein binds to extracellular matrices independently of collagen.
P2860
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P2860
Variant von Willebrand's disease: characterization of two subtypes by analysis of multimeric composition of factor VIII/von Willebrand factor in plasma and platelets.
description
article científic
@ca
article scientifique
@fr
articolo scientifico
@it
artigo científico
@pt
bilimsel makale
@tr
scientific article published on June 1980
@en
vedecký článok
@sk
vetenskaplig artikel
@sv
videnskabelig artikel
@da
vědecký článek
@cs
name
Variant von Willebrand's disea ...... actor in plasma and platelets.
@en
Variant von Willebrand's disea ...... actor in plasma and platelets.
@nl
type
label
Variant von Willebrand's disea ...... actor in plasma and platelets.
@en
Variant von Willebrand's disea ...... actor in plasma and platelets.
@nl
prefLabel
Variant von Willebrand's disea ...... actor in plasma and platelets.
@en
Variant von Willebrand's disea ...... actor in plasma and platelets.
@nl
P2860
P356
P1476
Variant von Willebrand's disea ...... actor in plasma and platelets.
@en
P2093
T S Zimmerman
Z M Ruggeri
P2860
P304
P356
10.1172/JCI109795
P407
P577
1980-06-01T00:00:00Z