ISTH 2022 Congress

Platelet Disorders, von Willebrand Disease and Thrombotic Microangiopathies » von Willebrand Factor Biology

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ISTH 2022 Congress
- Platelet Disorders, von Willebrand Disease and Thrombotic Microangiopathies
  - von Willebrand Factor Biology

Abstract Number: PB0804

A new type 1 von Willebrand Disease (VWD) characterized by increased clearance of von Willebrand Factor (VWF) due to the heterozygous p.P1127S mutation: clinical phenotype and pathogenic mechanisms.

M. Tardugno¹, M. Sacco¹, R. De Cristofaro¹, S. Lancellotti², F. Bernardi³, M. Pinotti³, A. Branchini⁴, B. Giusti⁵, G. Castaman⁶

Abstract Number: PB1433

Analysis of uncharacterised Von Willebrand’s Disease causing mutations located in the D4 and C-domains: identification of a novel type 2A variant and gain-of-function VWF

G. Mobayen¹, M. Appiah², M. Laffan³, T. McKinnon⁴

Abstract Number: OC 22.1

Angiopoietin-2 binds to Von Willebrand Factor within Endothelial Cells and after Release from Weibel Palade Bodies and mediates Von Willebrand Factor dependent angiogenesis

T. McKinnon¹, R. Starke², K. Smith², G. Mobayen², K. Paschalaki³, A. Randi¹, M. Laffan⁴

Abstract Number: PB0351

Blood transfusion maintains endothelial cell quiescence – implications in the prevention of stroke in paediatric sickle cell anaemia

H. Fogarty¹, S. Ward², E. Karampini², S. Elliott³, A. Rehill², A. Ahmad², J. Velasquez², R. Geoghegan⁴, H. Conroy⁴, M. Byrne⁵, U. Budde⁶, E. Tuohy⁷, C. McMahon⁴, J. O'Donnell²

Abstract Number: PB0796

Characterisation of 17 Novel Von Willebrand Factor Missense Mutations

M. Appiah¹, A. Shaida², G. Mobayen², T. McKinnon³

Abstract Number: PB0801

Conformational dynamics of plasma-derived von Willebrand factor recapitulate isolated A1 domain and autoinhibitory module

E. Legan¹, M. Wilson², N. Arce¹, E. Parker², P. Lollar², R. Li¹

Abstract Number: OC 53.2

Deep mutational scan of the VWF C domains to define mutations associated with VWD

T. Sparring¹, H. Madarati², K. Singh³, C. Kretz²

Abstract Number: OC 43.5

Domain-specific nanobodies distinguish intact and proteolyzed forms of von Willebrand factor in congenital von Willebrand disease

C. Kizlik-Masson¹, I. Peyron², S. Gangnard³, M. Clavel³, V. Regnault⁴, E. Jeanpierre⁵, A. Dupont⁶, O. Christophe³, C. Casari⁷, C. Denis³, S. Susen⁸, P. Lenting³

Abstract Number: PB0798

Evaluation of thrombin generation capacity of plasma-derived Factor VIII/von Willebrand Factor in the Treatment of von Willebrand Disease

P. Lafuente¹, M. Bravo², M. Costa², T. Barnett³, T. Willis³

Abstract Number: OC 43.1

Idntification of a von Willebrand factor homozygous deep intronic variant by next-generation analysis, and subsequent validation by mRNA analysis and study of the patient-derived endothelial cells

H. Yadegari¹, M. Jamil², J. Oldenburg³

Abstract Number: OC 22.2

Inhibition of von Willebrand factor through stabilization of the ristocetin-binding site

N. Arce, R. Li

Abstract Number: PB0799

Multi-scale modeling of SIPA showing VWF agglomeration and capture of platelets with high shear

D. Ku¹, Z. Lu², C. Bresette³, C. Aidun⁴

Abstract Number: OC 53.4

O-linked glycosylation of VWF modulates Weibel-Palade body formation and VWF secretion

E. Karampini¹, S. Elliott², S. Ward³, J. O'Sullivan⁴, I. Schoen⁵, J. O'Donnell³

Abstract Number: OC 43.4

Plasmin-cleaved VWF: a biomarker for microthrombosis

H. El Otmani¹, S. Smits¹, N. van Kleef¹, R. Frunt¹, S. De Maat², C. Maas²

Abstract Number: PB0800

Quantitative von Willebrand factor multimer analysis in von Willebrand disease patients enrolled in the Zimmerman Program study.

H. Clift¹, P. Christopherson², V. Flood³, K. Friedman⁴, R. Montgomery⁵, S. Haberichter⁵

Abstract Number: PB0797