Results 91 to 100 of about 56,156 (204)
Autosomal dominant C1149R von Willebrand disease: phenotypic findings and their implications
Haematologica, 2009 Background Mutation C1149R in the von Willebrand factor (VWF) gene has been thought to cause autosomal dominant severe type 1 von Willebrand disease (VWD).Design and Methods Eight patients from three unrelated families with this mutation were included in
Almudena Pérez-Rodríguez +5 more
doaj +1 more source
Enhanced VWF biosynthesis and elevated plasma VWF due to a natural variant in the murine Vwf gene [PDF]
Both genetic and environmental influences contribute to the wide variation in plasma von Willebrand factor (VWF) levels observed in humans. Inbred mouse strains also have highly variable plasma VWF levels, providing a convenient model in which to study ...
Lemmerhirt, Heidi L.
core
Effect of vWF on Notch signaling. [PDF]
, 2013 A co-culture system was used to assess the effects of vWF on Notch signaling in H460 (A) and A7R5 (B) cells reflected by ligand stimulation of HES-luciferase. Experiments were performed in either serum-containing or serum-free media supplemented with vWF
He Meng (33363) +3 more
core +1 more source
Haematologica, 2010 Background Type 2B von Willebrand factor (VWF) is characterized by gain of function mutations in the A1 domain inducing a greater affinity for platelet GPIb, possibly associated with the disappearance of large VWF multimers and thrombocytopenia.Design ...
Alessandra Casonato +7 more
doaj +1 more source
Identification of a VWF gene mutation. [PDF]
, 2013 Sequence analysis of the VWF gene in the proband detected a 6-bp nucleotides deletion in exon 28. The mutation caused D1529V1530 deletion (ΔD1529V1530) in VWF A2 domain. The ADAMTS13 cleavage site is indicated by an arrow.
Ningzheng Dong (328283) +7 more
core +1 more source
VWF cleavage by endothelial cell lysates depicted by immunoblotting. [PDF]
, 2013 A. Recombinant ADAMTS13 cleaves the A2 domain of VWF whereby 176 kDa and 140 kDa bands appear (lane 1). Full-length VWF is depicted by an arrow. Cell buffer, incubated with VWF and used as the negative control, did not exhibit the cleavage fragments ...
David Motto (349293) +8 more
core +1 more source
Thrombosis JournalBackground Von Willebrand factor (vWF) plays a crucial role in hemostasis, acting as a key factor for platelet adhesion/aggregation and as a transport protein for coagulation factor VIII. vWF is secreted as a giant multimer, and it undergoes shear stress-
Yuki Chiba +4 more
doaj +1 more source
Schematic domain structure of full-length VWF and recombinant VWF fragments. [PDF]
, 2013 The domain structure of human preproVWF is shown above the structures of recombinant VWF fragments designed in this study. ADAMTS-13 cleaves the Y1605-M1606 peptidyl bond in the A2 domain (D1459-L1668).
Ningzheng Dong (328283) +8 more
core +1 more source
GrM disrupts FVIII binding to VWF. [PDF]
, 2013 (A) Purified plasma VWF (1 µg/mL) VWF was incubated with indicated concentration of GrM, GrB, GrM-SA or GrB-SA in Tris (pH 7.4) for 1 hour at 37°C and subsequent FVIII binding was assessed in an ELISA setup.
Martine J. Hollestelle (345019) +6 more
core +1 more source
Research and Practice in Thrombosis and HaemostasisBackground: Transplant-associated thrombotic microangiopathy (TA-TMA) is a fatal complication of hematopoietic stem cell transplantation and is characterized by severe thrombocytopenia, hemolytic anemia, and organ dysfunction.
Shinya Yamada +6 more
doaj +1 more source