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Unique interactions of asialo von Willebrand factor with platelets in platelet-type von Willebrand disease
| Content Provider | Scilit |
|---|---|
| Author | Miller, Jl Ruggeri, Zm Lyle, Va |
| Copyright Year | 1987 |
| Description | 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. |
| Related Links | https://ashpublications.org/blood/article-pdf/70/6/1804/365675/1804.pdf |
| Ending Page | 1809 |
| Page Count | 6 |
| Starting Page | 1804 |
| DOI | 10.1182/blood.v70.6.1804.bloodjournal7061804 |
| Journal | Blood |
| Issue Number | 6 |
| Volume Number | 70 |
| Language | English |
| Publisher | American Society of Hematology |
| Publisher Date | 1987-12-01 |
| Access Restriction | Open |
| Subject Keyword | Hematology Antibody Edta Gpib Von Willebrand Prp Vwf Micrograms/ml Patient Platelets Journal: Blood (Vol- 88, Issue- 6) |
| Content Type | Text |
| Resource Type | Article |