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Titolo:
Biosynthesis, processing and secretion of von Willebrand factor: biological implications
Autore:
de Wit, TR; van Mourik, JA;
Indirizzi:
CLB, Dept Plasma Prot & Blood Coagulat, Amsterdam, Netherlands CLB Amsterdam Netherlands Prot & Blood Coagulat, Amsterdam, Netherlands Univ Amsterdam, Acad Med Ctr, Dept Vasc Med, NL-1105 AZ Amsterdam, Netherlands Univ Amsterdam Amsterdam Netherlands NL-1105 AZ Z Amsterdam, Netherlands
Titolo Testata:
BEST PRACTICE & RESEARCH CLINICAL HAEMATOLOGY
fascicolo: 2, volume: 14, anno: 2001,
pagine: 241 - 255
SICI:
1521-6926(200106)14:2<241:BPASOV>2.0.ZU;2-V
Fonte:
ISI
Lingua:
ENG
Soggetto:
WEIBEL-PALADE BODIES; HUMAN-ENDOTHELIAL-CELLS; RECEPTOR-RELATED PROTEIN; HUMAN VONWILLEBRAND-FACTOR; GRANULE MEMBRANE-PROTEIN; VIII-BINDING DOMAIN; FACTOR PROPEPTIDE; PLASMINOGEN-ACTIVATOR; PRO-POLYPEPTIDE; MULTIPLE ROLES;
Keywords:
von Willebrand factor; von Willebrand factor propeptide; factor VIII; Weibel-Palade bodies; endothelial cells;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
94
Recensione:
Indirizzi per estratti:
Indirizzo: van Mourik, JA CLB, Dept Plasma Prot & Blood Coagulat, Amsterdam, Netherlands CLB Amsterdam Netherlands agulat, Amsterdam, Netherlands
Citazione:
T.R. de Wit e J.A. van Mourik, "Biosynthesis, processing and secretion of von Willebrand factor: biological implications", BEST P R C, 14(2), 2001, pp. 241-255

Abstract

von Willebrand factor is a multimeric plasma glycoprotein that is requiredfor normal haemostasis. von Willebrand factor is synthesized by endothelial cells and megakaryocytes, and originates from its precursor pro-von Willebrand factor. The endoproteolytic processing of pro-von Willebrand factor results in mature von Willebrand factor and von Willebrand factor propeptide(also known as von Willebrand Ag II). In endothelial cells, the propeptidecontrols the polymerization and subsequent targeting of von Willebrand factor to the storage vesicles, the so-called Weibel-Palade bodies. Upon stimulation of the endothelial cells, the Weibel-Palade bod ies are translocatedto the plasma membrane of the cell, and mature von Willebrand factor and its propeptide are co-secreted. After release, these polypeptides have divergent fates and serve different biological functions. Mature von Willebrand factor both controls platelet adhesion and aggregation at sites of vascularinjury and acts as a chaperone protein for coagulation factor VIII. The von Willebrand factor propeptide may serve a role in modulating inflammatory processes. This still growing body of information indicates that the biological function of the von Willebrand factor gene product is more diverse than was previously thought.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 09/04/20 alle ore 00:20:44