PMID-sentid Pub_year Sent_text comp_official_name comp_offsetprotein_name organism prot_offset 33326604-3 2021 Importantly however, studies in other microangiopathies have shown that plasma VWF propeptide (VWFpp) is a more sensitive and specific measure of acute EC activation. propeptide 83-93 von Willebrand factor Homo sapiens 79-82 2784791-7 1989 Considering the fact that mature vWF supports platelet adhesion to subendothelium, present observations suggest that the propeptide portion and the mature protein could have opposing effects on hemostasis. propeptide 121-131 von Willebrand factor Homo sapiens 33-36 3034486-5 1986 Together, these molecular biology and protein chemistry studies have shown that vWAgII is the amino-terminal propeptide of vWF, explaining the proportional deficiency of these two plasma proteins in von Willebrand disease (type I). propeptide 109-119 von Willebrand factor Homo sapiens 123-126 2584182-8 1989 The signal peptide and propeptide (von Willebrand antigen II) of von Willebrand factor are encoded by 17 exons in approximately 80 kilobases of DNA while the mature subunit of von Willebrand factor and 3" noncoding region are encoded by 35 exons in the remaining approximately 100 kilobases of the gene. propeptide 23-33 von Willebrand factor Homo sapiens 65-86 26759371-0 2017 Plasma ADAMTS13, von Willebrand Factor (VWF), and VWF Propeptide Profiles in Patients With Connective Tissue Diseases and Antiphospholipid Syndrome. propeptide 54-64 von Willebrand factor Homo sapiens 50-53 31350267-4 2019 We observed that VWF:Ag and VWF propeptide (VWFpp)/VWF:Ag, but not VWFpp, were associated with FVIII half-life. propeptide 32-42 von Willebrand factor Homo sapiens 28-31 31350267-4 2019 We observed that VWF:Ag and VWF propeptide (VWFpp)/VWF:Ag, but not VWFpp, were associated with FVIII half-life. propeptide 32-42 von Willebrand factor Homo sapiens 28-31 29992156-4 2018 The ratio between the VWF propeptide and the mature VWF antigen is used to diagnose these patients. propeptide 26-36 von Willebrand factor Homo sapiens 22-25 29992156-5 2018 Commercial VWF propeptide assays are too expensive for use in developing countries. propeptide 15-25 von Willebrand factor Homo sapiens 11-14 29992156-7 2018 Methods: We first displayed VWF propeptide on yeast. propeptide 32-42 von Willebrand factor Homo sapiens 28-31 29992156-8 2018 Antibody fragments were selected against the displayed VWF propeptide by using phage display technology. propeptide 59-69 von Willebrand factor Homo sapiens 55-58 29992156-9 2018 The antibodies were used to develop a cost-effective VWF propeptide assay and compared to a commercial VWF propeptide assay. propeptide 57-67 von Willebrand factor Homo sapiens 53-56 29992156-10 2018 Results: Two of these antibody fragments bound specific to the VWF propeptide and not to the yeast used for the expression of the propeptides. propeptide 67-77 von Willebrand factor Homo sapiens 63-66 29992156-11 2018 These purified antibody fragments were able to detect VWF propeptide in normal plasma. propeptide 58-68 von Willebrand factor Homo sapiens 54-57 29992156-13 2018 It also showed a higher binding affinity for VWF propeptide in plasma at especially lower plasma concentrations. propeptide 49-59 von Willebrand factor Homo sapiens 45-48 28916584-7 2017 In contrast, VWF propeptide to VWF:Ag ratios >3 were observed in only 6% of the LoVIC cohort. propeptide 17-27 von Willebrand factor Homo sapiens 13-16 28916584-7 2017 In contrast, VWF propeptide to VWF:Ag ratios >3 were observed in only 6% of the LoVIC cohort. propeptide 17-27 von Willebrand factor Homo sapiens 31-34 32705883-0 2020 Plasma Level of von Willebrand Factor Propeptide at Diagnosis: A Marker of Subsequent Renal Dysfunction in Autoimmune Rheumatic Diseases. propeptide 38-48 von Willebrand factor Homo sapiens 16-37 29913537-1 2019 An increased von Willebrand factor propeptide (VWFpp) to VWF antigen (VWF:Ag) ratio (VWFpp/VWF:Ag) indicates an enhanced clearance of VWF. propeptide 35-45 von Willebrand factor Homo sapiens 47-50 30290394-7 2018 It is known that factor VIII (FVIII) and von Willebrand factor (VWF) increase after exercise, but we found that this increase in FVIII and VWF (antigen, propeptide, and VWF in active conformation) was smaller on each of three subsequent days, suggesting either adaptation of endothelial activation or exhaustion of endothelial VWF supplies. propeptide 153-163 von Willebrand factor Homo sapiens 139-142 30290394-7 2018 It is known that factor VIII (FVIII) and von Willebrand factor (VWF) increase after exercise, but we found that this increase in FVIII and VWF (antigen, propeptide, and VWF in active conformation) was smaller on each of three subsequent days, suggesting either adaptation of endothelial activation or exhaustion of endothelial VWF supplies. propeptide 153-163 von Willebrand factor Homo sapiens 139-142 30290394-7 2018 It is known that factor VIII (FVIII) and von Willebrand factor (VWF) increase after exercise, but we found that this increase in FVIII and VWF (antigen, propeptide, and VWF in active conformation) was smaller on each of three subsequent days, suggesting either adaptation of endothelial activation or exhaustion of endothelial VWF supplies. propeptide 153-163 von Willebrand factor Homo sapiens 139-142 27344059-5 2016 SUMMARY: Background A variant of von Willebrand disease (VWD) type 2A, phenotype IIC (VWD2AIIC), is characterized by recessive inheritance, low von Willebrand factor antigen (VWF:Ag), lack of VWF high-molecular-weight multimers, absence of VWF proteolytic fragments and mutations in the VWF propeptide. propeptide 291-301 von Willebrand factor Homo sapiens 144-165 28320178-1 2017 INTRODUCTION: Von Willebrand factor propeptide (VWF:Ag II) is proposed to be a more sensitive marker of acute endothelial activation than von Willebrand factor antigen (VWF:Ag). propeptide 36-46 von Willebrand factor Homo sapiens 48-51 26346441-7 2017 Although the ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 domain, member 13) levels were markedly reduced, von Willebrand factor (VWF) and VWF propeptide (VWFpp) were markedly elevated during LDLT. propeptide 173-183 von Willebrand factor Homo sapiens 169-172 28296884-8 2017 The high levels of VWF in melioidosis appeared to be due to increased endothelial stimulation (VWF propeptide levels were elevated, p<0.0001) and reduced clearance (ADAMTS13 reduction, p<0.0001). propeptide 99-109 von Willebrand factor Homo sapiens 19-22 27344059-5 2016 SUMMARY: Background A variant of von Willebrand disease (VWD) type 2A, phenotype IIC (VWD2AIIC), is characterized by recessive inheritance, low von Willebrand factor antigen (VWF:Ag), lack of VWF high-molecular-weight multimers, absence of VWF proteolytic fragments and mutations in the VWF propeptide. propeptide 291-301 von Willebrand factor Homo sapiens 175-178 27344059-5 2016 SUMMARY: Background A variant of von Willebrand disease (VWD) type 2A, phenotype IIC (VWD2AIIC), is characterized by recessive inheritance, low von Willebrand factor antigen (VWF:Ag), lack of VWF high-molecular-weight multimers, absence of VWF proteolytic fragments and mutations in the VWF propeptide. propeptide 291-301 von Willebrand factor Homo sapiens 192-195 27344059-5 2016 SUMMARY: Background A variant of von Willebrand disease (VWD) type 2A, phenotype IIC (VWD2AIIC), is characterized by recessive inheritance, low von Willebrand factor antigen (VWF:Ag), lack of VWF high-molecular-weight multimers, absence of VWF proteolytic fragments and mutations in the VWF propeptide. propeptide 291-301 von Willebrand factor Homo sapiens 192-195 27344059-5 2016 SUMMARY: Background A variant of von Willebrand disease (VWD) type 2A, phenotype IIC (VWD2AIIC), is characterized by recessive inheritance, low von Willebrand factor antigen (VWF:Ag), lack of VWF high-molecular-weight multimers, absence of VWF proteolytic fragments and mutations in the VWF propeptide. propeptide 291-301 von Willebrand factor Homo sapiens 192-195 27344059-15 2016 A decreased ratio of VWF propeptide to VWF:Ag and a 1-desamino-8-d-arginine vasopressin (DDAVP) test in one patient indicated delayed VWF clearance, which was supported by clearance data after infusion of rmVWF into VWF(-/-) mice. propeptide 25-35 von Willebrand factor Homo sapiens 21-24 27359253-0 2016 Genome-wide studies of von Willebrand factor propeptide identify loci contributing to variation in propeptide levels and von Willebrand factor clearance. propeptide 45-55 von Willebrand factor Homo sapiens 23-44 27359253-0 2016 Genome-wide studies of von Willebrand factor propeptide identify loci contributing to variation in propeptide levels and von Willebrand factor clearance. propeptide 45-55 von Willebrand factor Homo sapiens 121-142 27359253-0 2016 Genome-wide studies of von Willebrand factor propeptide identify loci contributing to variation in propeptide levels and von Willebrand factor clearance. propeptide 99-109 von Willebrand factor Homo sapiens 23-44 27359253-2 2016 We performed a genome-wide association study of plasma VWF propeptide in 3,238 individuals. propeptide 59-69 von Willebrand factor Homo sapiens 55-58 27359253-7 2016 VWF propeptide (VWFpp) and mature VWF are encoded by the VWF gene and secreted at the same rate, but have different plasma half-lives. propeptide 4-14 von Willebrand factor Homo sapiens 0-3 27359253-7 2016 VWF propeptide (VWFpp) and mature VWF are encoded by the VWF gene and secreted at the same rate, but have different plasma half-lives. propeptide 4-14 von Willebrand factor Homo sapiens 16-19 27359253-7 2016 VWF propeptide (VWFpp) and mature VWF are encoded by the VWF gene and secreted at the same rate, but have different plasma half-lives. propeptide 4-14 von Willebrand factor Homo sapiens 16-19 26215113-3 2015 VWF is synthesized with a very large propeptide (VWFpp) that is critical for intracellular processing of VWF. propeptide 37-47 von Willebrand factor Homo sapiens 0-3 26215113-3 2015 VWF is synthesized with a very large propeptide (VWFpp) that is critical for intracellular processing of VWF. propeptide 37-47 von Willebrand factor Homo sapiens 49-52 26339184-1 2015 Epidemiological studies have shown that vascular-related disorders are associated with high von Willebrand factor antigen (VWF:Ag) and VWF propeptide (VWFpp). propeptide 139-149 von Willebrand factor Homo sapiens 135-138 25846964-6 2015 An increase in VWF propeptide levels was paralleled by a decrease in ADAMTS13 activity. propeptide 19-29 von Willebrand factor Homo sapiens 15-18 25876231-11 2015 VWF:propeptide, ADAMTS-13 and elastase levels were normal in ET patients. propeptide 4-14 von Willebrand factor Homo sapiens 0-3 25522671-8 2015 RESULTS: Following DDAVP administration, VWF, FVIII, and VWF propeptide levels increased in patients with haemophilia, while patients with cirrhosis only showed an increase in VWF propeptide and FVIII levels. propeptide 61-71 von Willebrand factor Homo sapiens 57-60 25522671-8 2015 RESULTS: Following DDAVP administration, VWF, FVIII, and VWF propeptide levels increased in patients with haemophilia, while patients with cirrhosis only showed an increase in VWF propeptide and FVIII levels. propeptide 61-71 von Willebrand factor Homo sapiens 57-60 26088471-0 2015 Mutations in the D1 domain of von Willebrand factor impair their propeptide-dependent multimerization, intracellular trafficking and secretion. propeptide 65-75 von Willebrand factor Homo sapiens 30-51 26088471-1 2015 We identified three novel mutations (p.Gly39Arg, p.Lys157Glu, p.Cys379Gly) and one previously known mutation (p.Asp141Asn) in the von Willebrand factor propeptide from three von Willebrand disease patients. propeptide 152-162 von Willebrand factor Homo sapiens 130-151 26088471-2 2015 All four mutations impaired multimerization of von Willebrand factor, due to reduced oxidoreductase activity of isomeric propeptide. propeptide 121-131 von Willebrand factor Homo sapiens 47-68 25953977-0 2015 VWF propeptide in defining VWD subtypes. propeptide 4-14 von Willebrand factor Homo sapiens 0-3 24951428-3 2014 VWF propeptide (VWFpp) was elevated with an infinitely high VWFpp to VWF:Ag ratio (VWFpp:Ag) consistent with rapid VWF clearance. propeptide 4-14 von Willebrand factor Homo sapiens 0-3 25860876-8 2015 In archived clinical samples, the activity of ADAMTS13 in septic patients treated with APC (n = 5) increased with an accompanying decrease in VWF propeptide as surrogate for improved endothelial function. propeptide 146-156 von Willebrand factor Homo sapiens 142-145 25215616-5 2015 Her activated partial thromboplastin time was slightly prolonged, the ratio between von Willebrand factor (vWF) propeptide and vWF antigen was in the normal range, but the ratio between vWF and ristocetin cofactor was decreased dramatically. propeptide 112-122 von Willebrand factor Homo sapiens 107-110 24951428-3 2014 VWF propeptide (VWFpp) was elevated with an infinitely high VWFpp to VWF:Ag ratio (VWFpp:Ag) consistent with rapid VWF clearance. propeptide 4-14 von Willebrand factor Homo sapiens 16-19 24951428-3 2014 VWF propeptide (VWFpp) was elevated with an infinitely high VWFpp to VWF:Ag ratio (VWFpp:Ag) consistent with rapid VWF clearance. propeptide 4-14 von Willebrand factor Homo sapiens 16-19 22120183-8 2013 Total active VWF and VWF propeptide correlated with the period of being diagnosed with diabetes. propeptide 25-35 von Willebrand factor Homo sapiens 21-24 23702511-5 2013 Candidate VWF mutations were identified in 25 of 26 IC and included propeptide missense mutations in four IC (two resulting in type 1 and two in recessive 2A), all influencing VWF expression in vitro. propeptide 68-78 von Willebrand factor Homo sapiens 176-179 23481506-3 2013 We evaluated the association between the VWF-propeptide (VWF-pp)/ADAMTS13 ratio and disease severity in patients with severe sepsis or septic shock. propeptide 45-55 von Willebrand factor Homo sapiens 41-44 23481506-3 2013 We evaluated the association between the VWF-propeptide (VWF-pp)/ADAMTS13 ratio and disease severity in patients with severe sepsis or septic shock. propeptide 45-55 von Willebrand factor Homo sapiens 57-60 23349392-0 2013 VWF propeptide and ratios between VWF, VWF propeptide, and FVIII in the characterization of type 1 von Willebrand disease. propeptide 4-14 von Willebrand factor Homo sapiens 0-3 23349392-1 2013 During posttranslational modifications of von Willebrand factor (VWF), the VWF propeptide (VWFpp) is cleaved. propeptide 79-89 von Willebrand factor Homo sapiens 42-63 23349392-1 2013 During posttranslational modifications of von Willebrand factor (VWF), the VWF propeptide (VWFpp) is cleaved. propeptide 79-89 von Willebrand factor Homo sapiens 65-68 23349392-1 2013 During posttranslational modifications of von Willebrand factor (VWF), the VWF propeptide (VWFpp) is cleaved. propeptide 79-89 von Willebrand factor Homo sapiens 75-78 23266519-2 2013 RESULTS: ADAMTS13 activities were slightly low, and plasma levels of VWF and VWF propeptide (VWFpp) antigens and the ratio of VWFpp/VWF were significantly high before LDLT. propeptide 81-91 von Willebrand factor Homo sapiens 77-80 23266519-2 2013 RESULTS: ADAMTS13 activities were slightly low, and plasma levels of VWF and VWF propeptide (VWFpp) antigens and the ratio of VWFpp/VWF were significantly high before LDLT. propeptide 81-91 von Willebrand factor Homo sapiens 77-80 21352469-8 2011 Both VWF antigen and VWF propeptide levels were significantly higher in patients with mild and moderate aortic stenosis, but not in those with severe stenosis. propeptide 25-35 von Willebrand factor Homo sapiens 21-24 24182550-6 2013 vWF and vWF propeptide were significantly elevated in patients with sickle cell disease; this was more pronounced in patients with the HbSS/HbSbeta genotype. propeptide 12-22 von Willebrand factor Homo sapiens 8-11 22452980-0 2012 von Willebrand factor (VWF) propeptide binding to VWF D"D3 domain attenuates platelet activation and adhesion. propeptide 28-38 von Willebrand factor Homo sapiens 0-21 22452980-0 2012 von Willebrand factor (VWF) propeptide binding to VWF D"D3 domain attenuates platelet activation and adhesion. propeptide 28-38 von Willebrand factor Homo sapiens 23-26 22452980-0 2012 von Willebrand factor (VWF) propeptide binding to VWF D"D3 domain attenuates platelet activation and adhesion. propeptide 28-38 von Willebrand factor Homo sapiens 50-53 22452980-1 2012 Noncovalent association between the von Willebrand factor (VWF) propeptide (VWFpp) and mature VWF aids N-terminal multimerization and protein compartmentalization in storage granules. propeptide 64-74 von Willebrand factor Homo sapiens 36-57 22452980-1 2012 Noncovalent association between the von Willebrand factor (VWF) propeptide (VWFpp) and mature VWF aids N-terminal multimerization and protein compartmentalization in storage granules. propeptide 64-74 von Willebrand factor Homo sapiens 59-62 22452980-1 2012 Noncovalent association between the von Willebrand factor (VWF) propeptide (VWFpp) and mature VWF aids N-terminal multimerization and protein compartmentalization in storage granules. propeptide 64-74 von Willebrand factor Homo sapiens 76-79 22070827-0 2012 Plasma ADAMTS13, von Willebrand factor (VWF) and VWF propeptide profiles in patients with DIC and related diseases. propeptide 53-63 von Willebrand factor Homo sapiens 49-52 22563509-6 2012 There were significant differences in the kinetics of the various WPB constituents: VWF propeptide and OPG levels decreased toward discharge, while VWF:Ag levels were lower than expected at enrollment with plasma levels increasing toward discharge. propeptide 88-98 von Willebrand factor Homo sapiens 84-87 22563509-7 2012 Moreover, VWF propeptide levels correlated better with markers of disease severity (platelet count, liver enzymes, serum albumin and pleural effusion index) than corresponding VWF levels. propeptide 14-24 von Willebrand factor Homo sapiens 10-13 22102187-0 2011 Assessment of von Willebrand factor propeptide improves the diagnosis of von Willebrand disease. propeptide 36-46 von Willebrand factor Homo sapiens 14-35 22102187-3 2011 An alternative now available involves assaying the VWF propeptide (VWFpp) in single steady-state blood samples, which reportedly increases as VWF survival decreases. propeptide 55-65 von Willebrand factor Homo sapiens 51-54 22102187-3 2011 An alternative now available involves assaying the VWF propeptide (VWFpp) in single steady-state blood samples, which reportedly increases as VWF survival decreases. propeptide 55-65 von Willebrand factor Homo sapiens 67-70 22102202-7 2011 From the type 1 VWD patients who were diagnosed by the VWD testing facility, 45% seem to have an increased VWF clearance phenotype with a propeptide-to-antigen ratio of 1.9 +- 0.3. propeptide 138-148 von Willebrand factor Homo sapiens 107-110 20335223-5 2010 We identified an N528S homozygous mutation in the VWF propeptide D2 domain, predicting the introduction of an additional N-glycosylation site at amino acid 526 in close vicinity to a "CGLC" disulphide isomerase consensus sequence. propeptide 54-64 von Willebrand factor Homo sapiens 50-53 21153061-0 2011 Elevated Von Willebrand factor propeptide for the diagnosis of thrombotic microangiopathy and for predicting a poor outcome. propeptide 31-41 von Willebrand factor Homo sapiens 9-30 21153061-2 2011 Von Willebrand factor (VWF) propeptide (VWFpp) is considered to be a marker of vascular endothelial cell injury. propeptide 28-38 von Willebrand factor Homo sapiens 0-21 21153061-2 2011 Von Willebrand factor (VWF) propeptide (VWFpp) is considered to be a marker of vascular endothelial cell injury. propeptide 28-38 von Willebrand factor Homo sapiens 23-26 20305138-5 2010 The similarly reduced VWF half-life was also confirmed by the increase in the VWF propeptide ratio (a useful tool for exploring VWF survival) which was found to be the same in type 2B and atypical type 2B patients. propeptide 82-92 von Willebrand factor Homo sapiens 22-25 20305138-5 2010 The similarly reduced VWF half-life was also confirmed by the increase in the VWF propeptide ratio (a useful tool for exploring VWF survival) which was found to be the same in type 2B and atypical type 2B patients. propeptide 82-92 von Willebrand factor Homo sapiens 78-81 20305138-5 2010 The similarly reduced VWF half-life was also confirmed by the increase in the VWF propeptide ratio (a useful tool for exploring VWF survival) which was found to be the same in type 2B and atypical type 2B patients. propeptide 82-92 von Willebrand factor Homo sapiens 78-81 20403097-4 2010 Rare alleles of a-245c, G12E, and S331S, which were in linkage disequilibrium, were associated with higher VWF propeptide, VWF and FVIII levels. propeptide 111-121 von Willebrand factor Homo sapiens 107-110 20403097-7 2010 The binding affinity of V2R for AVP was increased three-fold in 12E-V2R-green fluorescent protein (GFP) cells, which is in accordance with increased levels of VWF propeptide associated with the 12E variant. propeptide 163-173 von Willebrand factor Homo sapiens 159-162 20403097-10 2010 CONCLUSIONS: The 12E-V2R variant has increased binding affinity for AVP, resulting in increased signal transduction, and is associated with increased levels of VWF propeptide, VWF, and FVIII. propeptide 164-174 von Willebrand factor Homo sapiens 160-163 20335223-0 2010 The mutation N528S in the von Willebrand factor (VWF) propeptide causes defective multimerization and storage of VWF. propeptide 54-64 von Willebrand factor Homo sapiens 26-47 20335223-0 2010 The mutation N528S in the von Willebrand factor (VWF) propeptide causes defective multimerization and storage of VWF. propeptide 54-64 von Willebrand factor Homo sapiens 49-52 20335223-0 2010 The mutation N528S in the von Willebrand factor (VWF) propeptide causes defective multimerization and storage of VWF. propeptide 54-64 von Willebrand factor Homo sapiens 113-116 20590859-4 2010 This phenotype can be suspected by the presence of an increased ratio between the VWF propeptide and the VWF antigen. propeptide 86-96 von Willebrand factor Homo sapiens 82-85 20335223-9 2010 In addition, we have identified a potentially novel pathogenic mechanism of VWD, namely a transportation and storage defect of mature VWF due to defective interaction with its transporter, the mutant propeptide. propeptide 200-210 von Willebrand factor Homo sapiens 134-137 20158601-5 2010 RESULTS: At admission, all patients had increased levels of (active) VWF and VWF propeptide. propeptide 81-91 von Willebrand factor Homo sapiens 77-80 20158601-6 2010 The highest VWF propeptide levels were observed in patients with shock, indicating acute endothelial activation. propeptide 16-26 von Willebrand factor Homo sapiens 12-15 19192112-1 2009 BACKGROUND: The large von Willebrand factor (VWF) propeptide (VWFpp) plays a critical role in the multimerization and regulated storage of the mature VWF protein. propeptide 50-60 von Willebrand factor Homo sapiens 22-43 20303469-5 2010 The VWF-propeptide-to-VWF-antigen (VWF:Ag) ratio (VWFpp ratio) was higher in patients with a shorter VWF survival, and the values were inversely correlated with the VWF half-life (P<0.01). propeptide 8-18 von Willebrand factor Homo sapiens 4-7 20303469-5 2010 The VWF-propeptide-to-VWF-antigen (VWF:Ag) ratio (VWFpp ratio) was higher in patients with a shorter VWF survival, and the values were inversely correlated with the VWF half-life (P<0.01). propeptide 8-18 von Willebrand factor Homo sapiens 22-25 20303469-5 2010 The VWF-propeptide-to-VWF-antigen (VWF:Ag) ratio (VWFpp ratio) was higher in patients with a shorter VWF survival, and the values were inversely correlated with the VWF half-life (P<0.01). propeptide 8-18 von Willebrand factor Homo sapiens 22-25 20303469-5 2010 The VWF-propeptide-to-VWF-antigen (VWF:Ag) ratio (VWFpp ratio) was higher in patients with a shorter VWF survival, and the values were inversely correlated with the VWF half-life (P<0.01). propeptide 8-18 von Willebrand factor Homo sapiens 22-25 20303469-5 2010 The VWF-propeptide-to-VWF-antigen (VWF:Ag) ratio (VWFpp ratio) was higher in patients with a shorter VWF survival, and the values were inversely correlated with the VWF half-life (P<0.01). propeptide 8-18 von Willebrand factor Homo sapiens 22-25 19578117-4 2009 On the other hand, loop 2 of IL-8 closely resembles a surface-exposed sequence of the VWF propeptide, the region of VWF that directs sorting of the protein to WPB. propeptide 90-100 von Willebrand factor Homo sapiens 86-89 19578117-4 2009 On the other hand, loop 2 of IL-8 closely resembles a surface-exposed sequence of the VWF propeptide, the region of VWF that directs sorting of the protein to WPB. propeptide 90-100 von Willebrand factor Homo sapiens 116-119 19707594-3 2009 METHODOLOGY: Standardized half-life studies and analysis of pre-infusion VWF and VWF-propeptide levels were performed in a cohort of 38 patients with severe haemophilia A (FVIII <1 IU/ml), aged 15-44 years. propeptide 85-95 von Willebrand factor Homo sapiens 81-84 19707594-5 2009 Using multivariate linear regression-analysis (MVLR-analysis), the association of VWF-antigen, VWF-propeptide, age and body-weight with FVIII half-life was evaluated. propeptide 99-109 von Willebrand factor Homo sapiens 95-98 19308894-5 2009 The potential use of supplementary assays such as the PFA-100 and the VWF propeptide assay after DDAVP challenge is also worth noting. propeptide 74-84 von Willebrand factor Homo sapiens 70-73 19192112-1 2009 BACKGROUND: The large von Willebrand factor (VWF) propeptide (VWFpp) plays a critical role in the multimerization and regulated storage of the mature VWF protein. propeptide 50-60 von Willebrand factor Homo sapiens 45-48 19192112-1 2009 BACKGROUND: The large von Willebrand factor (VWF) propeptide (VWFpp) plays a critical role in the multimerization and regulated storage of the mature VWF protein. propeptide 50-60 von Willebrand factor Homo sapiens 62-65 19506357-3 2009 Recessive VWD type 2C (2A subtype IIC) is caused by homozygosity or double heterozygosity of missense mutations in the D1 and D2 domains of the VWF propeptide (pp) that catalyzes the multimerization in the D3 domain at the N terminus of mature VWF. propeptide 148-158 von Willebrand factor Homo sapiens 144-147 19506357-3 2009 Recessive VWD type 2C (2A subtype IIC) is caused by homozygosity or double heterozygosity of missense mutations in the D1 and D2 domains of the VWF propeptide (pp) that catalyzes the multimerization in the D3 domain at the N terminus of mature VWF. propeptide 148-158 von Willebrand factor Homo sapiens 244-247 19506359-12 2009 Recent studies showed that the ratio of VWF propeptide (pp) to VWF:Ag can be used to predict a shorter than normal half-life for VWF:Ag. propeptide 44-54 von Willebrand factor Homo sapiens 40-43 18344424-0 2008 Identification of type 1 von Willebrand disease patients with reduced von Willebrand factor survival by assay of the VWF propeptide in the European study: molecular and clinical markers for the diagnosis and management of type 1 VWD (MCMDM-1VWD). propeptide 121-131 von Willebrand factor Homo sapiens 117-120 18691167-0 2008 Von Willebrand factor propeptide makes it easy to identify the shorter Von Willebrand factor survival in patients with type 1 and type Vicenza von Willebrand disease. propeptide 22-32 von Willebrand factor Homo sapiens 0-21 18691167-0 2008 Von Willebrand factor propeptide makes it easy to identify the shorter Von Willebrand factor survival in patients with type 1 and type Vicenza von Willebrand disease. propeptide 22-32 von Willebrand factor Homo sapiens 71-92 18691167-2 2008 The usefulness of VWF propeptide (VWFpp) in exploring VWF half-life was assessed in 22 type 1 and 14 type Vicenza VWD patients, and in 30 normal subjects, by comparing the findings on post-Desmopressin (DDAVP) VWF t(1/2) elimination (t(1/2el)). propeptide 22-32 von Willebrand factor Homo sapiens 34-37 18691167-2 2008 The usefulness of VWF propeptide (VWFpp) in exploring VWF half-life was assessed in 22 type 1 and 14 type Vicenza VWD patients, and in 30 normal subjects, by comparing the findings on post-Desmopressin (DDAVP) VWF t(1/2) elimination (t(1/2el)). propeptide 22-32 von Willebrand factor Homo sapiens 34-37 18690339-0 2008 Characterization of a novel mutation in the von Willebrand factor propeptide in a distinct subtype of recessive von Willebrand disease. propeptide 66-76 von Willebrand factor Homo sapiens 44-65 18328061-0 2008 Expression studies of missense mutations p.D141Y, p.C275S located in the propeptide of von Willebrand factor in patients with type 3 von Willebrand disease. propeptide 73-83 von Willebrand factor Homo sapiens 87-108 18245665-6 2008 The VWF propeptide to VWF:Ag ratio, useful for predicting an increased VWF clearance, was found significantly higher in O than in non-O individuals (1.6+/-0.1 vs 1.2+/-0.5, P<.001), with values that correlated inversely with T1/2el (P<.001). propeptide 8-18 von Willebrand factor Homo sapiens 4-7 18245665-6 2008 The VWF propeptide to VWF:Ag ratio, useful for predicting an increased VWF clearance, was found significantly higher in O than in non-O individuals (1.6+/-0.1 vs 1.2+/-0.5, P<.001), with values that correlated inversely with T1/2el (P<.001). propeptide 8-18 von Willebrand factor Homo sapiens 22-25 18245665-6 2008 The VWF propeptide to VWF:Ag ratio, useful for predicting an increased VWF clearance, was found significantly higher in O than in non-O individuals (1.6+/-0.1 vs 1.2+/-0.5, P<.001), with values that correlated inversely with T1/2el (P<.001). propeptide 8-18 von Willebrand factor Homo sapiens 22-25 18344424-2 2008 We have previously reported that the ratio of plasma levels of VWF and its propeptide (VWFpp) can be used to identify patients with reduced VWF survival. propeptide 75-85 von Willebrand factor Homo sapiens 87-90 18344424-9 2008 The systematic assay of both plasma VWF and the VWF propeptide in moderately severe type 1 VWD patients may identify patients with a reduced VWF survival phenotype. propeptide 52-62 von Willebrand factor Homo sapiens 48-51 18344424-9 2008 The systematic assay of both plasma VWF and the VWF propeptide in moderately severe type 1 VWD patients may identify patients with a reduced VWF survival phenotype. propeptide 52-62 von Willebrand factor Homo sapiens 48-51 17456630-5 2007 Twenty subjects were heterozygous for the R854Q mutation, one was heterozygous for the R760C missense mutation, which interferes with cleavage of the VWF propeptide. propeptide 154-164 von Willebrand factor Homo sapiens 150-153 17949477-2 2008 The ratio of the VWF propeptide (VWFpp) to VWF:Ag and the ratio of coagulation factor VIII (FVIII:C) to VWF:Ag have previously been used as indicators of VWF clearance and/or secretion. propeptide 21-31 von Willebrand factor Homo sapiens 17-20 17764538-6 2007 However, a contribution of acute endothelial dysfunction to renal impairment in sepsis is suggested by the significantly higher VWF propeptide and soluble thrombomodulin levels in patients with increased creatinine values as well as by their strong positive correlations (creatinine and VWF propeptide r(s) = 0.484, P < 0.001; creatinine and soluble thrombomodulin r(s) = 0.596, P < 0.001). propeptide 132-142 von Willebrand factor Homo sapiens 128-131 17895385-2 2007 This unusual oxidoreductase reaction requires the VWF propeptide (domains D1D2), which acts as an endogenous pH-dependent chaperone. propeptide 54-64 von Willebrand factor Homo sapiens 50-53 16953269-3 2006 This patient is heterozygous for both the frequent R854Q type 2NVWD mutation and a novel R763G mutation at the cleavage site between VWF propeptide and mature VWF. propeptide 137-147 von Willebrand factor Homo sapiens 133-136 17090649-9 2007 Of interest, a linear relation between PNA binding and propeptide/VWF ratio was observed (Spearman rank = 0.47), suggesting a potential association between O-linked glycosylation and VWF survival. propeptide 55-65 von Willebrand factor Homo sapiens 183-186 17059421-4 2006 VWF is secreted in equimolar amounts with its propeptide, which has a shorter half-life. propeptide 46-56 von Willebrand factor Homo sapiens 0-3 17059421-5 2006 VWF propeptide can be used as a measure of VWF secretion and allows estimation of the VWF half-life. propeptide 4-14 von Willebrand factor Homo sapiens 0-3 17059421-5 2006 VWF propeptide can be used as a measure of VWF secretion and allows estimation of the VWF half-life. propeptide 4-14 von Willebrand factor Homo sapiens 43-46 17059421-5 2006 VWF propeptide can be used as a measure of VWF secretion and allows estimation of the VWF half-life. propeptide 4-14 von Willebrand factor Homo sapiens 43-46 17059421-7 2006 In controls, high VWF propeptide was associated with high VWF:Ag, FVIII:Ag and FVIII:C. In contrast to mature VWF:Ag, VWF propeptide was not influenced by blood groups. propeptide 22-32 von Willebrand factor Homo sapiens 18-21 17059421-7 2006 In controls, high VWF propeptide was associated with high VWF:Ag, FVIII:Ag and FVIII:C. In contrast to mature VWF:Ag, VWF propeptide was not influenced by blood groups. propeptide 22-32 von Willebrand factor Homo sapiens 58-61 17059421-7 2006 In controls, high VWF propeptide was associated with high VWF:Ag, FVIII:Ag and FVIII:C. In contrast to mature VWF:Ag, VWF propeptide was not influenced by blood groups. propeptide 22-32 von Willebrand factor Homo sapiens 58-61 17059421-7 2006 In controls, high VWF propeptide was associated with high VWF:Ag, FVIII:Ag and FVIII:C. In contrast to mature VWF:Ag, VWF propeptide was not influenced by blood groups. propeptide 22-32 von Willebrand factor Homo sapiens 58-61 17059421-8 2006 Using an ELISA-based assay we have shown that VWF propeptide lacks ABO antigens. propeptide 50-60 von Willebrand factor Homo sapiens 46-49 17059421-11 2006 VWF propeptide was higher in thrombosis patients than in controls. propeptide 4-14 von Willebrand factor Homo sapiens 0-3 17139363-5 2006 The small transcript derives from the skipping of exon 14, the long one from the activation of a cryptic splice site in intron 13; both show a premature stop codon in VWF propeptide, so the proband VWF derives entirely from the correct splice site recognition. propeptide 171-181 von Willebrand factor Homo sapiens 167-170 17139363-5 2006 The small transcript derives from the skipping of exon 14, the long one from the activation of a cryptic splice site in intron 13; both show a premature stop codon in VWF propeptide, so the proband VWF derives entirely from the correct splice site recognition. propeptide 171-181 von Willebrand factor Homo sapiens 198-201 16835381-0 2006 Assay of the von Willebrand factor (VWF) propeptide to identify patients with type 1 von Willebrand disease with decreased VWF survival. propeptide 41-51 von Willebrand factor Homo sapiens 13-34 16835381-0 2006 Assay of the von Willebrand factor (VWF) propeptide to identify patients with type 1 von Willebrand disease with decreased VWF survival. propeptide 41-51 von Willebrand factor Homo sapiens 36-39 16835381-0 2006 Assay of the von Willebrand factor (VWF) propeptide to identify patients with type 1 von Willebrand disease with decreased VWF survival. propeptide 41-51 von Willebrand factor Homo sapiens 123-126 16835381-5 2006 A decreased survival of VWF in affected individuals was identified with VWF half-lives of 1 to 3 hours, whereas the half-life of VWF propeptide (VWFpp) was normal. propeptide 133-143 von Willebrand factor Homo sapiens 24-27 17200766-9 2007 Postoperative VWF:Ag and VWF-propeptide levels significantly increased in both groups. propeptide 29-39 von Willebrand factor Homo sapiens 25-28 16968329-11 2006 The propeptide/mature VWF ratio was increased 1.7-fold compared with healthy pregnant volunteers (P < 0.001) and 1.5-fold compared with patients with pre-eclampsia (P < 0.05). propeptide 4-14 von Willebrand factor Homo sapiens 22-25 16953269-8 2006 We conclude that R763G is a new type 2N VWD mutation located in the VWF propeptide which alters the proteolytic processing of VWF and consequently its binding to FVIII. propeptide 72-82 von Willebrand factor Homo sapiens 68-71 16953269-8 2006 We conclude that R763G is a new type 2N VWD mutation located in the VWF propeptide which alters the proteolytic processing of VWF and consequently its binding to FVIII. propeptide 72-82 von Willebrand factor Homo sapiens 126-129 16681646-0 2006 von Willebrand factor propeptide in malaria: evidence of acute endothelial cell activation. propeptide 22-32 von Willebrand factor Homo sapiens 0-21 16681646-4 2006 Our data show that acute endothelial cell activation is a hallmark of malaria in children, indicated by a significant rise in VWF and VWF propeptide. propeptide 138-148 von Willebrand factor Homo sapiens 134-137 16681646-5 2006 The highest VWF and propeptide levels were seen in cerebral and non-cerebral severe malaria, and associations found between VWF propeptide level and lactate (P < 0.001). propeptide 128-138 von Willebrand factor Homo sapiens 124-127 16681646-6 2006 Mean VWF propeptide levels (nmol/l) were in cerebral malaria 33.4, non-cerebral severe malaria 26.3, mild malaria 22.1, non-malaria febrile illness 10.2, and controls 10.1. propeptide 9-19 von Willebrand factor Homo sapiens 5-8 16681646-8 2006 Follow-up of 26 cerebral malaria cases showed that levels of VWF propeptide, but not VWF fell by 24 h, following the clinical course of disease and recovery. propeptide 65-75 von Willebrand factor Homo sapiens 61-64 12786801-2 2003 We reassessed these reference values using different assays, and those of the propeptide (VWF:Ag II) and factor VIII coagulant activity (factor VIII:C), in a large population of normal pregnancies, at 3-week intervals of gestational age. propeptide 78-88 von Willebrand factor Homo sapiens 90-93 16459301-4 2006 Further, while the propeptide and the N-terminal domains of mature VWF are sufficient to form tubules, their maintenance relies on a pH-dependent interaction between the two. propeptide 19-29 von Willebrand factor Homo sapiens 67-70 16102036-3 2005 von Willebrand factor multimer assembly depends on the ability of the propeptide to promote disulfide bond formation in the Golgi, possibly by acting as a pH-sensitive oxidoreductase. propeptide 70-80 von Willebrand factor Homo sapiens 0-21 14526622-10 2003 VWF propeptide, activated partial thromboplastin time and factor VIII are of importance. propeptide 4-14 von Willebrand factor Homo sapiens 0-3 14690496-7 2004 Unusually, the VWF propeptide is implicated in the biogenesis of WPBs, being essential for formation of the storage compartment. propeptide 19-29 von Willebrand factor Homo sapiens 15-18 12595584-8 2003 During the follow-up, a significant increase of plasma vWF and its propeptide has been observed in the group of patients who later developed microalbuminuria but not in those who remained normoalbuminuric. propeptide 67-77 von Willebrand factor Homo sapiens 55-58 12618268-0 2003 Differential intracellular trafficking of von Willebrand factor (vWF) and vWF propeptide in porcine endothelial cells lacking Weibel-Palade bodies and in human endothelial cells. propeptide 78-88 von Willebrand factor Homo sapiens 74-77 12595584-11 2003 In conclusion, an increase in plasma concentration of vWF and its propeptide precedes microalbuminuria and, therefore, can be useful to identify children with insulin-dependent diabetes mellitus at risk to develop incipient nephropathy later in life. propeptide 66-76 von Willebrand factor Homo sapiens 54-57 12393513-0 2003 Critical independent regions in the VWF propeptide and mature VWF that enable normal VWF storage. propeptide 40-50 von Willebrand factor Homo sapiens 36-39 12393698-0 2003 An Arg760Cys mutation in the consensus sequence of the von Willebrand factor propeptide cleavage site is responsible for a new von Willebrand disease variant. propeptide 77-87 von Willebrand factor Homo sapiens 55-76 12393698-1 2003 We describe a von Willebrand disease (VWD) variant characterized by the persistence of von Willebrand factor (VWF) propeptide as a result of a C>T transition at nucleotide 2527 in exon 17 of the VWF gene. propeptide 115-125 von Willebrand factor Homo sapiens 87-108 12393698-1 2003 We describe a von Willebrand disease (VWD) variant characterized by the persistence of von Willebrand factor (VWF) propeptide as a result of a C>T transition at nucleotide 2527 in exon 17 of the VWF gene. propeptide 115-125 von Willebrand factor Homo sapiens 110-113 12393698-1 2003 We describe a von Willebrand disease (VWD) variant characterized by the persistence of von Willebrand factor (VWF) propeptide as a result of a C>T transition at nucleotide 2527 in exon 17 of the VWF gene. propeptide 115-125 von Willebrand factor Homo sapiens 198-201 12393698-2 2003 This mutation, which was present in the proband and his father, predicts the substitution of Cys for Arg at position 760 of pre-pro-VWF, 4 residues before the propeptide cleavage site belonging to a consensus sequence for substrate recognition by the processing enzyme paired dibasic amino acid-cleaving enzyme (PACE)/furin. propeptide 159-169 von Willebrand factor Homo sapiens 132-135 12393734-3 2003 The VWF-related parameters in the volunteers increased 60 minutes after start of infusion by 3.7-fold for VWF:Ag, 7.2-fold for propeptide, and 2.2-fold for VWF:CBA. propeptide 127-137 von Willebrand factor Homo sapiens 4-7 12393698-6 2003 The persistence of VWF propeptide did not impair VWF synthesis because platelet VWF content was normal, nor did it compromise VWF storage in endothelial cells, because of the normal post-1-deamino-8-D-arginine vasopressin (DDAVP) increase in plasma VWF. propeptide 23-33 von Willebrand factor Homo sapiens 19-22 12393698-8 2003 These findings confirm that a normal consensus sequence for VWF propeptide cleavage and efficient cleavage are required in vivo for normal FVIII binding capacity of VWF. propeptide 64-74 von Willebrand factor Homo sapiens 60-63 12393698-8 2003 These findings confirm that a normal consensus sequence for VWF propeptide cleavage and efficient cleavage are required in vivo for normal FVIII binding capacity of VWF. propeptide 64-74 von Willebrand factor Homo sapiens 165-168 12393513-4 2003 The VWF propeptide (VWFpp) targets VWF to storage granules through a noncovalent association. propeptide 8-18 von Willebrand factor Homo sapiens 4-7 12393513-4 2003 The VWF propeptide (VWFpp) targets VWF to storage granules through a noncovalent association. propeptide 8-18 von Willebrand factor Homo sapiens 20-23 11299058-0 2001 Von Willebrand factor propeptide as a marker of disease activity in systemic sclerosis (scleroderma). propeptide 22-32 von Willebrand factor Homo sapiens 0-21 12176890-0 2002 The role of the D1 domain of the von Willebrand factor propeptide in multimerization of VWF. propeptide 55-65 von Willebrand factor Homo sapiens 33-54 12176890-0 2002 The role of the D1 domain of the von Willebrand factor propeptide in multimerization of VWF. propeptide 55-65 von Willebrand factor Homo sapiens 88-91 12176890-3 2002 This mutation in the propeptide (VWFpp) resulted in synthesis of dimeric VWF with an almost complete loss of N-terminal multimerization. propeptide 21-31 von Willebrand factor Homo sapiens 33-36 11776313-1 2001 Von Willebrand factor (vWF) is synthesized in endothelial cells as pre-provWF and processed intracellularly to propeptide (vWFpp) and mature vWF. propeptide 111-121 von Willebrand factor Homo sapiens 0-21 11776313-1 2001 Von Willebrand factor (vWF) is synthesized in endothelial cells as pre-provWF and processed intracellularly to propeptide (vWFpp) and mature vWF. propeptide 111-121 von Willebrand factor Homo sapiens 23-26 11487004-2 2001 In endothelial cells, processing of its precursor pro-VWF results in the formation of two large polypeptides, mature VWF and a propeptide. propeptide 127-137 von Willebrand factor Homo sapiens 54-57 11686098-4 2001 In endothelial cells, the propeptide controls the polymerization and subsequent targeting of von Willebrand factor to the storage vesicles, the so-called Weibel-Palade bodies. propeptide 26-36 von Willebrand factor Homo sapiens 93-114 11686098-8 2001 The von Willebrand factor propeptide may serve a role in modulating inflammatory processes. propeptide 26-36 von Willebrand factor Homo sapiens 4-25 9157601-0 1997 Acute von Willebrand factor secretion from the endothelium in vivo: assessment through plasma propeptide (vWf:AgII) Levels. propeptide 94-104 von Willebrand factor Homo sapiens 6-27 10896250-6 2000 whereas, the release of other granule proteins such as vWF-propeptide or serotonin remained unchanged. propeptide 59-69 von Willebrand factor Homo sapiens 55-58 10381511-1 1999 Before de novo synthesized von Willebrand factor (vWF) leaves the endothelial cell, it undergoes endoproteolytic cleavage of its propeptide (vW antigen II). propeptide 129-139 von Willebrand factor Homo sapiens 27-48 10381511-1 1999 Before de novo synthesized von Willebrand factor (vWF) leaves the endothelial cell, it undergoes endoproteolytic cleavage of its propeptide (vW antigen II). propeptide 129-139 von Willebrand factor Homo sapiens 50-53 10381511-3 1999 In a recent study (Borchiellini et al, Blood 88:2951, 1996), we showed that the half-life of mature vWF and of its propeptide differ fourfold to fivefold. propeptide 115-125 von Willebrand factor Homo sapiens 100-103 9869174-2 1998 vWf is stored and released from endothelial cell secretory granules, along with equimolar amounts of its propeptide (vWf:AgII). propeptide 105-115 von Willebrand factor Homo sapiens 0-3 9869174-2 1998 vWf is stored and released from endothelial cell secretory granules, along with equimolar amounts of its propeptide (vWf:AgII). propeptide 105-115 von Willebrand factor Homo sapiens 117-120 9869174-4 1998 vWf but not propeptide levels are influenced by blood groups, explaining in part the smaller variation in plasma propeptide levels among normal individuals. propeptide 113-123 von Willebrand factor Homo sapiens 0-3 9869174-5 1998 In both controls and insulin-dependent diabetic patients, we found a close correlation between propeptide and immunoreactive vWf levels (r2=0.54, p <0.0001). propeptide 95-105 von Willebrand factor Homo sapiens 125-128 9759633-1 1998 Measurement of the von Willebrand factor (vWF) propeptide, also known as von Willebrand antigen II, has been suggested to be helpful in the discrimination of congenital von Willebrand disease type I from type 2 and in assessing the extent of activation of the endothelium. propeptide 47-57 von Willebrand factor Homo sapiens 19-40 9759633-1 1998 Measurement of the von Willebrand factor (vWF) propeptide, also known as von Willebrand antigen II, has been suggested to be helpful in the discrimination of congenital von Willebrand disease type I from type 2 and in assessing the extent of activation of the endothelium. propeptide 47-57 von Willebrand factor Homo sapiens 42-45 9759633-4 1998 In contrast, propeptide levels were significantly higher in AvWS (11.4+/-1.1 vs 4.7+/-0.2 nM, p <0.001), probably reflecting a compensatory increase in vWF synthesis or increased perturbation of the endothelium in AvWS. propeptide 13-23 von Willebrand factor Homo sapiens 155-158 9759633-6 1998 Measurement of propeptide levels may provide additional information in AvWS as to whether decreased levels of mature vWF in the circulation are due to a decrease in synthesis or due to an accelerated removal of vWF from the circulation. propeptide 15-25 von Willebrand factor Homo sapiens 117-120 9714529-0 1998 A new candidate mutation (N528S) within the von Willebrand factor propeptide identified in a Japanese patient with phenotype IIC of von Willebrand disease. propeptide 66-76 von Willebrand factor Homo sapiens 44-65 9714529-4 1998 The propositus is homozygous for the A1833G nucleotide substitution, in exon 14 of vWF gene, responsible for the N528S mutation within the vWF propeptide. propeptide 143-153 von Willebrand factor Homo sapiens 83-86 9714529-4 1998 The propositus is homozygous for the A1833G nucleotide substitution, in exon 14 of vWF gene, responsible for the N528S mutation within the vWF propeptide. propeptide 143-153 von Willebrand factor Homo sapiens 139-142 9714529-8 1998 Furthermore, the N528S nonconservative substitution identified is located in the vWF propeptide region, where the other phenotype IIC mutations described so far are clustered. propeptide 85-95 von Willebrand factor Homo sapiens 81-84 9657753-7 1998 The requirement for vWF propeptide cleavage was shown by (1) a point mutation of the vWF propeptide cleavage site yielding pro-vWF that was defective in factor VIII binding and (2) correlation between efficiency of intracellular propeptide cleavage and factor VIII binding. propeptide 89-99 von Willebrand factor Homo sapiens 85-88 9657753-7 1998 The requirement for vWF propeptide cleavage was shown by (1) a point mutation of the vWF propeptide cleavage site yielding pro-vWF that was defective in factor VIII binding and (2) correlation between efficiency of intracellular propeptide cleavage and factor VIII binding. propeptide 89-99 von Willebrand factor Homo sapiens 85-88 9657753-9 1998 Our results indicate that high-affinity factor VIII binding sites are located on N-terminal disulfide-linked vWF subunits from which the propeptide has been cleaved. propeptide 137-147 von Willebrand factor Homo sapiens 109-112 10961880-1 2000 The von Willebrand factor propeptide, vW AgII, has been shown to be required for the formation of vWF multimers and sorting of vWF to storage granules; whether these 2 processes are independent events has been unclear. propeptide 26-36 von Willebrand factor Homo sapiens 98-101 10961880-1 2000 The von Willebrand factor propeptide, vW AgII, has been shown to be required for the formation of vWF multimers and sorting of vWF to storage granules; whether these 2 processes are independent events has been unclear. propeptide 26-36 von Willebrand factor Homo sapiens 127-130 9657753-0 1998 Binding of factor VIII to von willebrand factor is enabled by cleavage of the von Willebrand factor propeptide and enhanced by formation of disulfide-linked multimers. propeptide 100-110 von Willebrand factor Homo sapiens 26-47 9657753-0 1998 Binding of factor VIII to von willebrand factor is enabled by cleavage of the von Willebrand factor propeptide and enhanced by formation of disulfide-linked multimers. propeptide 100-110 von Willebrand factor Homo sapiens 78-99 9657753-2 1998 Prior reports suggest that posttranslational modifications of vWF, including formation of N-terminal intersubunit disulfide bonds and subsequent cleavage of the propeptide, influence availability and/or affinity of factor VIII binding sites. propeptide 161-171 von Willebrand factor Homo sapiens 62-65 9657753-3 1998 We found that deletion of the vWF propeptide produced a dimeric vWF molecule lacking N-terminal intersubunit disulfide bonds. propeptide 34-44 von Willebrand factor Homo sapiens 30-33 9657753-3 1998 We found that deletion of the vWF propeptide produced a dimeric vWF molecule lacking N-terminal intersubunit disulfide bonds. propeptide 34-44 von Willebrand factor Homo sapiens 64-67 9657753-5 1998 Coexpression of propeptide-deleted vWF with the vWF propeptide in trans yielded multimeric vWF that displayed increased affinity for factor VIII. propeptide 16-26 von Willebrand factor Homo sapiens 35-38 9657753-5 1998 Coexpression of propeptide-deleted vWF with the vWF propeptide in trans yielded multimeric vWF that displayed increased affinity for factor VIII. propeptide 16-26 von Willebrand factor Homo sapiens 48-51 9657753-5 1998 Coexpression of propeptide-deleted vWF with the vWF propeptide in trans yielded multimeric vWF that displayed increased affinity for factor VIII. propeptide 16-26 von Willebrand factor Homo sapiens 48-51 9657753-5 1998 Coexpression of propeptide-deleted vWF with the vWF propeptide in trans yielded multimeric vWF that displayed increased affinity for factor VIII. propeptide 52-62 von Willebrand factor Homo sapiens 35-38 9657753-5 1998 Coexpression of propeptide-deleted vWF with the vWF propeptide in trans yielded multimeric vWF that displayed increased affinity for factor VIII. propeptide 52-62 von Willebrand factor Homo sapiens 48-51 9657753-5 1998 Coexpression of propeptide-deleted vWF with the vWF propeptide in trans yielded multimeric vWF that displayed increased affinity for factor VIII. propeptide 52-62 von Willebrand factor Homo sapiens 48-51 9657753-7 1998 The requirement for vWF propeptide cleavage was shown by (1) a point mutation of the vWF propeptide cleavage site yielding pro-vWF that was defective in factor VIII binding and (2) correlation between efficiency of intracellular propeptide cleavage and factor VIII binding. propeptide 24-34 von Willebrand factor Homo sapiens 20-23 9657753-7 1998 The requirement for vWF propeptide cleavage was shown by (1) a point mutation of the vWF propeptide cleavage site yielding pro-vWF that was defective in factor VIII binding and (2) correlation between efficiency of intracellular propeptide cleavage and factor VIII binding. propeptide 24-34 von Willebrand factor Homo sapiens 85-88 9657753-7 1998 The requirement for vWF propeptide cleavage was shown by (1) a point mutation of the vWF propeptide cleavage site yielding pro-vWF that was defective in factor VIII binding and (2) correlation between efficiency of intracellular propeptide cleavage and factor VIII binding. propeptide 24-34 von Willebrand factor Homo sapiens 85-88 9657753-7 1998 The requirement for vWF propeptide cleavage was shown by (1) a point mutation of the vWF propeptide cleavage site yielding pro-vWF that was defective in factor VIII binding and (2) correlation between efficiency of intracellular propeptide cleavage and factor VIII binding. propeptide 89-99 von Willebrand factor Homo sapiens 20-23 9657753-7 1998 The requirement for vWF propeptide cleavage was shown by (1) a point mutation of the vWF propeptide cleavage site yielding pro-vWF that was defective in factor VIII binding and (2) correlation between efficiency of intracellular propeptide cleavage and factor VIII binding. propeptide 89-99 von Willebrand factor Homo sapiens 85-88 9657753-7 1998 The requirement for vWF propeptide cleavage was shown by (1) a point mutation of the vWF propeptide cleavage site yielding pro-vWF that was defective in factor VIII binding and (2) correlation between efficiency of intracellular propeptide cleavage and factor VIII binding. propeptide 89-99 von Willebrand factor Homo sapiens 85-88 9657753-7 1998 The requirement for vWF propeptide cleavage was shown by (1) a point mutation of the vWF propeptide cleavage site yielding pro-vWF that was defective in factor VIII binding and (2) correlation between efficiency of intracellular propeptide cleavage and factor VIII binding. propeptide 89-99 von Willebrand factor Homo sapiens 20-23 9684782-5 1998 The propeptide molar concentration was found to be 5 nM as compared to 31 nM for mature vWF. propeptide 4-14 von Willebrand factor Homo sapiens 88-91 9569179-0 1998 Ins405AsnPro mutation in the von Willebrand factor propeptide in recessive type 2A (IIC) von Willebrand"s disease. propeptide 51-61 von Willebrand factor Homo sapiens 29-50 9569179-2 1998 A six nucleotide insert, AATCCC, was found in exon 11 of the vWF gene, predicting the insertion of the amino acids asparagine and proline between phenylalanine 404 and threonine 405 of the vWF propeptide. propeptide 193-203 von Willebrand factor Homo sapiens 61-64 9569179-2 1998 A six nucleotide insert, AATCCC, was found in exon 11 of the vWF gene, predicting the insertion of the amino acids asparagine and proline between phenylalanine 404 and threonine 405 of the vWF propeptide. propeptide 193-203 von Willebrand factor Homo sapiens 189-192 9569179-8 1998 Taken together with those of earlier studies the present findings suggest that the IIC phenotype may well be exclusively caused by mutations which result in changes of the amino acid sequence in certain regions of the vWF propeptide. propeptide 222-232 von Willebrand factor Homo sapiens 218-221 9157601-0 1997 Acute von Willebrand factor secretion from the endothelium in vivo: assessment through plasma propeptide (vWf:AgII) Levels. propeptide 94-104 von Willebrand factor Homo sapiens 106-109 9157601-4 1997 The propeptide of vWf (also called vWf:AgII) is stored and released in equimolar amounts with vWf. propeptide 4-14 von Willebrand factor Homo sapiens 18-21 9157601-4 1997 The propeptide of vWf (also called vWf:AgII) is stored and released in equimolar amounts with vWf. propeptide 4-14 von Willebrand factor Homo sapiens 35-43 9157601-4 1997 The propeptide of vWf (also called vWf:AgII) is stored and released in equimolar amounts with vWf. propeptide 4-14 von Willebrand factor Homo sapiens 35-38 8857958-0 1996 A common frameshift mutation in von Willebrand factor does not alter mRNA stability but interferes with normal propeptide processing. propeptide 111-121 von Willebrand factor Homo sapiens 32-53 8874191-0 1996 Quantitative analysis of von Willebrand factor propeptide release in vivo: effect of experimental endotoxemia and administration of 1-deamino-8-D-arginine vasopressin in humans. propeptide 47-57 von Willebrand factor Homo sapiens 25-46 8874191-2 1996 Only fully processed, functionally mature vWF is stored within the cell, together with the propeptide, and leaves the cell only on stimulation (regulated secretion). propeptide 91-101 von Willebrand factor Homo sapiens 42-45 8874191-3 1996 Both in resting and stimulated cultured endothelial cells, the stoichiometry of the released propeptide to the released mature vWF is essentially equimolar. propeptide 93-103 von Willebrand factor Homo sapiens 127-130 8874191-6 1996 Our results show that the molar concentration of the propeptide in normal plasma is about one tenth of the concentration of mature vWF (expressed as half-dimer concentration). propeptide 53-63 von Willebrand factor Homo sapiens 131-134 8857958-8 1996 Consistent with these results, plasma VWF propeptide of the homozygous individual was markedly reduced whereas heterozygotes exhibited moderately reduced levels. propeptide 42-52 von Willebrand factor Homo sapiens 38-41 8800502-7 1996 In the rare subtype IIC, some aa substitutions or insertion have been found within the propeptide of vWF. propeptide 87-97 von Willebrand factor Homo sapiens 101-104 8049421-6 1994 Furthermore, the localization of these gene defects in the D2 domain of vWF propeptide, known to play an important role in vWF multimerization, provides another argument in favor of their causative effect regarding the peculiar multimeric pattern of vWF in these patients. propeptide 76-86 von Willebrand factor Homo sapiens 72-75 8049421-6 1994 Furthermore, the localization of these gene defects in the D2 domain of vWF propeptide, known to play an important role in vWF multimerization, provides another argument in favor of their causative effect regarding the peculiar multimeric pattern of vWF in these patients. propeptide 76-86 von Willebrand factor Homo sapiens 123-126 8049421-6 1994 Furthermore, the localization of these gene defects in the D2 domain of vWF propeptide, known to play an important role in vWF multimerization, provides another argument in favor of their causative effect regarding the peculiar multimeric pattern of vWF in these patients. propeptide 76-86 von Willebrand factor Homo sapiens 123-126 1571548-2 1992 By coexpression of vWF and various propeptide processing enzymes in COS-1 cells, we here demonstrate that vWF is preferentially processed by the paired dibasic amino acid-cleaving enzyme PACE (furin). propeptide 35-45 von Willebrand factor Homo sapiens 106-109 8324222-7 1993 By sequencing of cloned genomic DNA, mutations were excluded in exons 4, 5, 14, and 15, which encode regions of the vWF propeptide proposed to be important in multimer biosynthesis. propeptide 120-130 von Willebrand factor Homo sapiens 116-119 2265247-11 1990 Additionally, we have found that the cleaved propeptide of vWF is sulfated on asparagine-linked carbohydrate. propeptide 45-55 von Willebrand factor Homo sapiens 59-62 2251280-6 1990 The functional specificity of PACE for mediating propeptide cleavage at paired basic amino acid residues was demonstrated by the enhancement of propeptide processing of human von Willebrand factor when coexpressed with PACE in COS-1 cells. propeptide 49-59 von Willebrand factor Homo sapiens 175-196 2251280-6 1990 The functional specificity of PACE for mediating propeptide cleavage at paired basic amino acid residues was demonstrated by the enhancement of propeptide processing of human von Willebrand factor when coexpressed with PACE in COS-1 cells. propeptide 144-154 von Willebrand factor Homo sapiens 175-196 1939217-4 1991 The structural requirements for vWF propeptide cleavage and for vWF multimerization in its binding and stabilization of factor VIII was examined using specifically altered recombinant vWF. propeptide 36-46 von Willebrand factor Homo sapiens 32-35 1939217-6 1991 Deletion of the vWF propeptide produced a dimeric vWF molecule that failed to mediate platelet agglutination, suggesting that multimerization is required for vWF to attain functional GPIb binding. propeptide 20-30 von Willebrand factor Homo sapiens 16-19 1939217-6 1991 Deletion of the vWF propeptide produced a dimeric vWF molecule that failed to mediate platelet agglutination, suggesting that multimerization is required for vWF to attain functional GPIb binding. propeptide 20-30 von Willebrand factor Homo sapiens 50-53 1939217-6 1991 Deletion of the vWF propeptide produced a dimeric vWF molecule that failed to mediate platelet agglutination, suggesting that multimerization is required for vWF to attain functional GPIb binding. propeptide 20-30 von Willebrand factor Homo sapiens 50-53 1939217-8 1991 A vWF mutant with an altered propeptide cleavage site formed large multimers of uncleaved pro-vWF that functioned in platelet agglutination. propeptide 29-39 von Willebrand factor Homo sapiens 2-5 1939217-8 1991 A vWF mutant with an altered propeptide cleavage site formed large multimers of uncleaved pro-vWF that functioned in platelet agglutination. propeptide 29-39 von Willebrand factor Homo sapiens 94-97 1939217-10 1991 Analysis of the vWF propeptide, expressed independently, demonstrated that it could not bind factor VIII or stabilize its secretion. propeptide 20-30 von Willebrand factor Homo sapiens 16-19 1939217-11 1991 These results show that the dimeric mature vWF subunit is sufficient to bind and stabilize factor VIII and that the presence of uncleaved vWF propeptide inhibits both factor VIII binding and stabilization. propeptide 142-152 von Willebrand factor Homo sapiens 138-141 34940854-8 2021 The vWF propeptide/antigen ratio was 4:1, supporting a diagnosis of aVWD resulting from increased antibody-mediated vWF clearance. propeptide 8-18 von Willebrand factor Homo sapiens 4-7 33942438-0 2021 von Willebrand factor propeptide missense variants affect anterograde transport to Golgi resulting in ER retention. propeptide 22-32 von Willebrand factor Homo sapiens 0-21 33942438-2 2021 The present study investigated functional consequences and underlying pathomolecular mechanisms of several VWF propeptide (VWFpp) missense variants detected in our cohort of VWD patients for the first time. propeptide 111-121 von Willebrand factor Homo sapiens 107-110 33588457-0 2021 Functional Roles of the von Willebrand Factor Propeptide. propeptide 46-56 von Willebrand factor Homo sapiens 24-45 33588457-1 2021 The primary polypeptide sequence of von Willebrand factor (VWF) includes an N-terminal 741-amino acid VWF propeptide (VWFpp). propeptide 106-116 von Willebrand factor Homo sapiens 36-57 33588457-1 2021 The primary polypeptide sequence of von Willebrand factor (VWF) includes an N-terminal 741-amino acid VWF propeptide (VWFpp). propeptide 106-116 von Willebrand factor Homo sapiens 59-62 33588457-1 2021 The primary polypeptide sequence of von Willebrand factor (VWF) includes an N-terminal 741-amino acid VWF propeptide (VWFpp). propeptide 106-116 von Willebrand factor Homo sapiens 102-105 35466528-2 2022 The VWF propeptide is critical for multimerization and acts as an intra-molecular chaperone for mature VWF in sorting to its storage organelles, Weibel-Palade bodies (WPBs). propeptide 8-18 von Willebrand factor Homo sapiens 4-7 34409729-3 2021 METHODS: VWF antigen and VWF propeptide were measured in HIV negative participants (n=85), HIV infected virologically suppressed participants (n=89) and HIV infected participants with HIV viral load (VL) of >50 copies/ml (n=63) in each trimester. propeptide 29-39 von Willebrand factor Homo sapiens 25-28 34694218-4 2021 RESULTS: The patients studied showed significantly reduced VWF levels and function; an increased VWF propeptide to VWF antigen ratio; and all VWF multimers present but in reduced quantities, with the low-molecular-weight VWF forms being significantly more represented than those of higher molecular weight. propeptide 101-111 von Willebrand factor Homo sapiens 97-100 34409729-0 2021 von Willebrand Factor Propeptide to Antigen Ratio in HIV Infected Pregnancy: Evidence of Endothelial Activation. propeptide 22-32 von Willebrand factor Homo sapiens 0-21 34409729-2 2021 OBJECTIVES: To assess the state of endothelial activation in HIV infected pregnancy by measuring the von Willebrand factor (VWF) propeptide to antigen ratio, as an index of acute endothelial activation. propeptide 129-139 von Willebrand factor Homo sapiens 101-122 34409729-2 2021 OBJECTIVES: To assess the state of endothelial activation in HIV infected pregnancy by measuring the von Willebrand factor (VWF) propeptide to antigen ratio, as an index of acute endothelial activation. propeptide 129-139 von Willebrand factor Homo sapiens 124-127 34375505-9 2021 Importantly, EC biomarkers including VWF:Ag, VWF propeptide (VWFpp) and Factor VIII (FVIII:C) were significantly elevated in convalescent COVID-19 compared to controls (p=0.004, 95% CI 0.09-0.57IU/ml; p=0.009, 95% CI 0.06-0.5IU/ml; p=0.04, 95% CI 0.03-0.44IU/ml, respectively). propeptide 49-59 von Willebrand factor Homo sapiens 45-48 35148377-1 2022 The von Willebrand factor (VWF) propeptide (domains D1D2), is essential for the assembly of VWF multimers and its tubular storage in Weibel-Palade bodies. propeptide 32-42 von Willebrand factor Homo sapiens 4-25 35148377-1 2022 The von Willebrand factor (VWF) propeptide (domains D1D2), is essential for the assembly of VWF multimers and its tubular storage in Weibel-Palade bodies. propeptide 32-42 von Willebrand factor Homo sapiens 27-30 35148377-1 2022 The von Willebrand factor (VWF) propeptide (domains D1D2), is essential for the assembly of VWF multimers and its tubular storage in Weibel-Palade bodies. propeptide 32-42 von Willebrand factor Homo sapiens 92-95 35148377-7 2022 The clinically identified VWF mutations in the propeptide disrupted different steps of the assembling process leading to diminished VWF multimers in von Willebrand diseases (VWD). propeptide 47-57 von Willebrand factor Homo sapiens 26-29 35148377-7 2022 The clinically identified VWF mutations in the propeptide disrupted different steps of the assembling process leading to diminished VWF multimers in von Willebrand diseases (VWD). propeptide 47-57 von Willebrand factor Homo sapiens 132-135 35148377-8 2022 Overall, these results indicate that the propeptide serves as a pH sensing template for VWF multimerization and tubular storage. propeptide 41-51 von Willebrand factor Homo sapiens 88-91 35092343-0 2022 Von Willebrand factor propeptide and pathophysiological mechanisms in European and Iranian patients with type 3 von Willebrand disease enrolled in the 3WINTERS-IPS study. propeptide 22-32 von Willebrand factor Homo sapiens 0-21 35092343-2 2022 Pathophysiological mechanisms of VWD like defective synthesis, secretion and clearance of VWF have previously been evaluated using ratios of VWF propeptide (VWFpp) over VWF antigen (VWF:Ag) and factor (F)VIII coagulant activity (FVIII:C) over VWF:Ag. propeptide 145-155 von Willebrand factor Homo sapiens 90-93 35092343-2 2022 Pathophysiological mechanisms of VWD like defective synthesis, secretion and clearance of VWF have previously been evaluated using ratios of VWF propeptide (VWFpp) over VWF antigen (VWF:Ag) and factor (F)VIII coagulant activity (FVIII:C) over VWF:Ag. propeptide 145-155 von Willebrand factor Homo sapiens 141-144 35466528-2 2022 The VWF propeptide is critical for multimerization and acts as an intra-molecular chaperone for mature VWF in sorting to its storage organelles, Weibel-Palade bodies (WPBs). propeptide 8-18 von Willebrand factor Homo sapiens 103-106 35466528-3 2022 In the Canadian Type 3 VWD study, almost half of the identified variants were in the VWF propeptide and these were associated with an increased bleeding phenotype. propeptide 89-99 von Willebrand factor Homo sapiens 85-88 35466528-4 2022 OBJECTIVE: To investigate VWF propeptide variants that cause quantitative von Willebrand disease (VWD) by utilizing patient-derived endothelial colony-forming cells (ECFCs). propeptide 30-40 von Willebrand factor Homo sapiens 26-29 35466528-8 2022 In contrast, the other three propeptide variants presented a similar expression pattern in homozygous ECFCs where VWF was synthesized but not packaged in WPBs, and variant VWF had an increased association with the endoplasmic reticulum (ER) marker, protein disulfide-isomerase (PDI), indicating an ER-retention phenotype. propeptide 29-39 von Willebrand factor Homo sapiens 114-117 35466528-8 2022 In contrast, the other three propeptide variants presented a similar expression pattern in homozygous ECFCs where VWF was synthesized but not packaged in WPBs, and variant VWF had an increased association with the endoplasmic reticulum (ER) marker, protein disulfide-isomerase (PDI), indicating an ER-retention phenotype. propeptide 29-39 von Willebrand factor Homo sapiens 172-175 35466528-10 2022 CONCLUSION: This study further elucidates the importance of the VWF propeptide in the VWD phenotype using patient-derived cells. propeptide 68-78 von Willebrand factor Homo sapiens 64-67 35064738-0 2022 The dominant p.Thr274Pro mutation in the von Willebrand factor propeptide causes the von Willebrand disease type 1 phenotype in two unrelated patients. propeptide 63-73 von Willebrand factor Homo sapiens 41-62 35064738-1 2022 BACKGROUND: von Willebrand factor propeptide (VWFpp) plays an important role in VWF multimerization and storage. propeptide 34-44 von Willebrand factor Homo sapiens 12-33 35064738-1 2022 BACKGROUND: von Willebrand factor propeptide (VWFpp) plays an important role in VWF multimerization and storage. propeptide 34-44 von Willebrand factor Homo sapiens 80-83 35128300-4 2022 As a result, plasma levels of proteins normally stored within WPBs (including high-molecular-weight von Willebrand factor (VWF) multimers, VWF propeptide, and angiopoietin-2) are significantly elevated. propeptide 143-153 von Willebrand factor Homo sapiens 139-142