Clinical impact of glycans in platelet and megakaryocyte biology

H Falet, L Rivadeneyra… - Blood, The Journal of the …, 2022 - ashpublications.org
Humans produce and remove 1011 platelets daily to maintain a steady-state platelet count.
The tight regulation of platelet production and removal from the blood circulation prevents …

The Role of the Complement System in the Pathogenesis of Infectious Forms of Hemolytic Uremic Syndrome

PP Avdonin, MS Blinova, GA Generalova, KM Emirova… - Biomolecules, 2023 - mdpi.com
Hemolytic uremic syndrome (HUS) is an acute disease and the most common cause of
childhood acute renal failure. HUS is characterized by a triad of symptoms: microangiopathic …

Conformational activation and inhibition of von Willebrand factor by targeting its autoinhibitory module

NA Arce, Z Markham-Lee, Q Liang, S Najmudin… - Blood, 2024 - ashpublications.org
Activation of von Willebrand factor (VWF) is a tightly controlled process governed primarily
by local elements around its A1 domain. Recent studies suggest that the O-glycosylated …

Type 2B von Willebrand disease mutations differentially perturb autoinhibition of the A1 domain

ER Legan, Y Liu, NA Arce, ET Parker, P Lollar… - Blood, 2023 - ashpublications.org
Type 2B von Willebrand disease (VWD) is an inherited bleeding disorder in which a subset
of point mutations in the von Willebrand factor (VWF) A1 domain and recently identified …

O-glycan determinants regulate VWF trafficking to Weibel-Palade bodies

E Karampini, D Doherty, PE Bürgisser, M Garre… - Blood …, 2024 - ashpublications.org
Abstract von Willebrand factor (VWF) undergoes complex posttranslational modification
within endothelial cells (ECs) before secretion. This includes significant N-and O-linked …

[HTML][HTML] Autoinhibitory module underlies species difference in shear activation of von Willebrand factor

NA Arce, Y Liu, W Chen, XF Zhang, R Li - Journal of Thrombosis and …, 2022 - Elsevier
Abstract Background Von Willebrand factor (VWF) is a multimeric plasma protein that
bridges the gap between vessel injury and platelet capture at high shear rates. Under high …

Unraveling antibody-induced structural dynamics in the ADAMTS13 CUB1-2 domains via HDX-MS

Q Bonnez, ER Legan, I Pareyn, F Boudry, J Anckaert… - Blood Advances, 2025 - Elsevier
Allosteric regulation of ADAMTS13 (A Disintegrin And Metalloproteinase with
ThromboSpondin type-1 motif, member 13) activity involves an interaction between its …

2B or not 2B: art thou autoinhibitory?

TAJ McKinnon - Blood, The Journal of the American Society of …, 2023 - ashpublications.org
Just as the line from Hamlet “To be, or not to be—that is the question” is perhaps the most
famous Shakespearean quote, type 2B von Willebrand disease (VWD) is perhaps the most …

Structural studies of Von Willebrand Factor regulators for Thrombotic Thrombocytopenic Purpura.

ZJ Markham-Lee - 2024 - eprints.nottingham.ac.uk
Blood clotting requires an urgent and efficient response with von Willebrand factor (VWF)
playing a major role through recruitment of platelets forming a haemostatic plug. VWF is a …

[PDF][PDF] 2B or not 2B: art thou autoinhibitory?

T Uchiyama - Blood, 2023 - scholar.archive.org
Just as the line from Hamlet “To be, or not to be—that is the question” is perhaps the most
famous Shakespearean quote, type 2B von Willebrand disease (VWD) is perhaps the most …