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Unique interactions of asialo von Willebrand factor with platelets in platelet-type von Willebrand disease

Academic Article
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Overview

authors

  • Miller, J. L.
  • Ruggeri, Zaverio
  • Lyle, V. A.

publication date

  • December 1987

journal

  • Blood  Journal

abstract

  • The present studies demonstrate that platelets from patients with platelet-type von Willebrand disease show specific and saturable binding of asialo von Willebrand factor (AS-vWF) under conditions where such binding is not observed with normal platelets. Although specific binding of 125I-AS-vWF to formalin-fixed normal platelets could not be demonstrated, specific binding to fixed patient platelets was seen with an apparent Kd of 1.3 micrograms/mL and specific maximally bound ligand of 0.40 micrograms/10(8) platelets. Preincubation of patient platelets with the antiglycoprotein Ib (anti-GPIb) monoclonal antibody AS-2 reduced total binding close to the level of computer-estimated nonspecific binding. In contrast, binding was not reduced by preincubation with anti-GPIIb/IIIa monoclonal antibody or with 5 mmol/L EDTA. Under stirring conditions, the binding of AS-vWF to fixed patient platelets was accompanied by a strong agglutination response. AS-vWF-induced agglutination was similarly observed in patient but not normal platelet-rich plasma (PRP) in the presence of 5 mmol/L EDTA. In the absence of EDTA, AS-vWF produced a full aggregation response in patient PRP at concentrations as low as 0.1 microgram/mL in contrast to the 2 to 20 micrograms/mL required by normal PRP. Both thromboxane B2 formation and adenosine triphosphate secretion showed an AS-vWF concentration dependence paralleling the aggregation responses. These studies show that a major difference in the platelets from patients with platelet-type von Willebrand disease is the presence of an exposed, high-affinity binding site associated with GPIb that recognizes AS-vWF.

subject areas

  • Adenosine Triphosphate
  • Asialoglycoproteins
  • Binding Sites
  • Blood Platelets
  • Edetic Acid
  • Humans
  • Platelet Aggregation
  • Ristocetin
  • Thromboxane B2
  • von Willebrand Diseases
  • von Willebrand Factor
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Identity

International Standard Serial Number (ISSN)

  • 0006-4971

PubMed ID

  • 3118988
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Additional Document Info

start page

  • 1804

end page

  • 1809

volume

  • 70

issue

  • 6

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