von Willebrand factor propeptide: biology and clinical utility

摘要: von Willebrand factor (VWF) is a large multimeric glycoprotein that mediates the attachment of platelets to damaged endothelium and also serves as carrier protein for coagulation VIII (FVIII), protecting it from proteolytic degradation. Quantitative or qualitative defects in VWF result disease (VWD), common inherited bleeding disorder. synthesized with very propeptide (VWFpp) critical intracellular processing VWF. VWFpp actively participates process multimerization essential trafficking regulated storage pathway. Mutations identified within VWD patients are associated altered structure function. The assay plasma has clinical utility assessing acute chronic vascular perturbation diseases such thrombotic thrombocytopenic purpura, sepsis, diabetes among others. clear diagnosis subtypes, particularly discriminating true type 3 subjects 1C (reduced survival VWF), which clinically important implications therapeutic treatment.