Skip to main content

Funktionelle Charakterisierung von plasmatischem und rekombinantem von-Willebrand-Faktor durch Bindung an Collagen

  • Conference paper
28. Hämophilie-Symposion Hamburg 1997

Zusammenfassung

Dem von-Willebrand-Faktor (vWF) kommt neben seiner Bedeutung als Carrier und Stabilisator für Faktor VIII eine bedeutende Funktion in der primären Hämostase zu. Hier fungiert er als Bindeglied zwischen spezifischen Rezeptoren der Thrombozytenoberfläche und Komponenten der extrazellulären Matrix wie beispielsweise Collagen. Seine Domänenstruktur wird in Abbildung 1 wiedergegeben. Die Collagenbindungsepitope befinden sich innerhalb eines Disulfid-Loops der A1- bzw. A3-Domane der reifen Untereinheit des vWF [1].

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 44.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 59.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

Literatur

  1. Girma JP, Ribba AS, Meyer D (1995) Structure-function relationship of the Al domain of von Willebrand factor. Thromb Haemost 74: 156–160

    PubMed  CAS  Google Scholar 

  2. Weiss HJ,Hoyer LW,Rickles FR,Varma A, Rogers J (1973) Quantitative assay of a plasma factor deficient in von Willebrand’s disease that is necessary for platelet aggregation. Relationship to factor VIII procoagulant activity and antigen content. J Clin Invest 52: 2708–2716

    Article  PubMed  CAS  Google Scholar 

  3. Macfarlane DE, Stibbe J, Kirby EP, Zucker MB, Grant RA, McPherson J (1975) A method for assaying von Willebrand Factor ( Ristocetin Cofactor ). Thrombos Diathes haemorrh 34: 306–308

    CAS  Google Scholar 

  4. Brown JE, Bosak JO (1986) An ELISA test for the binding of von Willebrand antigen to collagen. Thromb Res 43: 303–311

    Article  PubMed  CAS  Google Scholar 

  5. Favaloro EJ, Grispo L, Exner T, Koutts J (1991) Development of a single collagen based ELISA assay aids in the diagnosis of, and permits sensitive discrimination between Type I and Type II, von Willebrand’s disease. Blood Coag Fibrinol 2: 285–291

    Article  CAS  Google Scholar 

  6. Thomas KB, Sutor AH, Zieger B, Jessat U, Grohmann A, Wendisch J, Budde U, von Kries R, Hasler K, Tune EP, Choong SC (1994) Ein einfacher Test fur die Bestimmung der Funktion des von-Willebrand-Faktors: die Kollagenbindungsaktivität.Hämostaseologie 14: 133–139

    CAS  Google Scholar 

  7. Pareti FI, Niiya K, McPherson JM, Ruggeri ZM (1987) Isolation and characterization of two domains of human von Willebrand Factor that interact with fibrillar collagen types I and III. J Biol Chem 262: 13835–13841

    PubMed  CAS  Google Scholar 

  8. de Groot PG, Ottenhof-Rovers M, van Mourik JA, Sixma JJ (1988) Evidence that the primary binding site of von Willebrand Factor that mediates platelet adhesion on subendothelium is not collagen. J Clin Invest 82: 65–73

    Article  PubMed  Google Scholar 

  9. Siekmann J, Turecek PL, Schwarz HP (1997) Design of a collagen binding assay for determination of the functional activity of von Willebrand Factor. Ann Hematol 74 ( Suppl. II ): A106

    Google Scholar 

  10. Fischer BE, Schlokat U, Mitterer A, Reiter A, Mundt W, Turecek PL, Schwarz HP, Dorner F (1995) Structural analysis of recombinant von Willebrand factor produced at industrial scale fermentation of transformed CHO cells co-expressing recombinant furin FEBS Lett. 375: 259–262

    CAS  Google Scholar 

  11. Sadler JE, Matsushita T, Dong ZY, Tuley EA, Westfield LA (1995) Molecular mechanism and classification of von Willebrand disease. Thromb Haemost 74: 161–166

    PubMed  CAS  Google Scholar 

  12. Ruggeri ZM, Zimmerman TS (1981) The complex multimeric composition of Factor VIII/ von Willebrand Factor. Blood 57: 1140–1143

    PubMed  CAS  Google Scholar 

  13. Auerswald G, Anders O, Budde U, Keller F, Kemkes-Matthes B, Kreibich U, Leithauser H, Marx G, Ponsel G, Scheel H, Schott G, Voss R, Wankmuller H (1995) Clinical study of the biological efficiency of a high-purity FVIII:C-vWF complex concentrate in patients with von Willebrand disease. Thromb Haemost 73: 1171

    Google Scholar 

  14. Furlan M, Robles R, Lammle B (1996) Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood 87: 4223–4234

    PubMed  CAS  Google Scholar 

  15. Turecek PL, Furlan M, Lämmle B, Richter G, Gritsch H, Siekmann J, Schwarz HP (1996) Cleavage of recombinant von Willebrand Factor (vWF) by a vWF-depolymerizing protease. Blood 88 (Suppl. 1): 326a

    Google Scholar 

  16. Aihara M, Sawada Y, Ueno K, Morimoto S, Yoshida Y, de Serres M, Cooper HA, Wagner RH (1986) Visualization of von Willebrand Factor multimers by immunoenzymatic stain using avidin-biotin peroxidase complex. Thromb Haemost 55: 263–267

    CAS  Google Scholar 

Download references

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 1999 Springer-Verlag Berlin Heidelberg

About this paper

Cite this paper

Siekmann, J., Turecek, P.L., Schwarz, H.P. (1999). Funktionelle Charakterisierung von plasmatischem und rekombinantem von-Willebrand-Faktor durch Bindung an Collagen. In: Scharrer, I., Schramm, W. (eds) 28. Hämophilie-Symposion Hamburg 1997. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-59915-6_18

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-59915-6_18

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-64581-8

  • Online ISBN: 978-3-642-59915-6

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics