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Unique interactions of asialo von Willebrand factor with platelets in platelet-type von Willebrand disease.
| Content Provider | Semantic Scholar |
|---|---|
| Author | Miller, Jonathan L. Ruggeri, Zaverio M. Lyle, V. A. |
| Copyright Year | 1987 |
| 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. |
| File Format | PDF HTM / HTML |
| DOI | 10.1182/blood.V70.6.1804.1804 |
| PubMed reference number | 3118988 |
| Journal | Medline |
| Volume Number | 70 |
| Issue Number | 6 |
| Alternate Webpage(s) | http://www.bloodjournal.org/content/bloodjournal/70/6/1804.full.pdf?sso-checked=true |
| Alternate Webpage(s) | https://doi.org/10.1182/blood.V70.6.1804.1804 |
| Journal | Blood |
| Language | English |
| Access Restriction | Open |
| Content Type | Text |
| Resource Type | Article |
