von Willebrand Disease Research This is an RSS file. You can use it to subscribe to this data in your favourite RSS reader or to display this data on your own website or blog.

Similar construction of spicules and shell plates: Implications for the origin of chiton biomineralization
In this study, we try to understand the types and diversity of matrix proteins in the biomineralization of chiton shell plates and spicules. Through a comparative analysis, we seek insights into the core biomineralization toolkit of ancestral mollusks. To achieve this, we conducted LC-MS/MS and RT-qPCR analyses to identify the types and relative expression levels of matrix proteins in both shell plates and spicules. The analysis revealed 96 matrix proteins in the spicules. A comparison of biomineralization-related matrix proteins in shell plates and spicules from the same species revealed shared proteins including many unk...
Source: Journal of Proteomics - February 16, 2024 Category: Biochemistry Authors: Haipeng Liu Chuang Liu Wenjing Zhang Yang Yuan Zhenglu Wang Jingliang Huang Source Type: research

Clusterin knockdown has effects on intracellular and secreted von Willebrand factor in human umbilical vein endothelial cells
by Allaura A. Cox, Alice Liu, Christopher J. Ng Alterations in von Willebrand factor (VWF) have an important role in human health and disease. Deficiency of VWF is associated with symptoms of bleeding and excesses of VWF are associated with thrombotic outcomes. Understanding the mechanisms that drive VWF regulation can lead to a better understa nding of modulation of VWF levels in humans. We identified clusterin (CLU) as a potential candidate regulator of VWF based on a single cell RNA sequencing (scRNA-seq) analysis in control endothelial cells (ECs) and von Willebrand disease (VWD) endothelial colony-forming-cells (ECFC...
Source: PLoS One - February 16, 2024 Category: Biomedical Science Authors: Allaura A. Cox Source Type: research

Efanesoctocog alfa: the renaissance of Factor VIII replacement therapy
Haematologica. 2024 Feb 15. doi: 10.3324/haematol.2023.284498. Online ahead of print.ABSTRACTEfanesoctocog alfa (ALTUVIIIOTM, Sanofi-SOBI) is a B domain-deleted single-chain Factor VIII (FVIII) connected to D'D3 domain of von Willebrand Factor (VWF). Its ingenious design allows efanesoctocog alfa to operate independently of endogenous VWF and results in an outstanding 3-4 times longer half-life compared to standard and extended half-life (EHL) FVIII products. The prolonged half-life ensures sustained high levels of factor activity, maintaining normal to near-normal ranges for the majority of the week, facilitating the conv...
Source: Haematologica - February 15, 2024 Category: Hematology Authors: Yesim Dargaud Alexandre Leuci Alejandra Reyes Ruiz Sebastien Lacroix-Desmazes Source Type: research

Mechanism underlying severe deficiency of plasma ADAMTS13 activity in immune thrombotic thrombocytopenic purpura
CONCLUSIONS: We conclude that severe deficiency of plasma ADAMTS13 activity is primarily resulted from antibody-mediated inhibition but the accelerated clearance of plasma ADAMTS13 antigen via immune complexes may also contribute significantly to severe deficiency of plasma ADAMTS13 activity in a subset of patients with acute iTTP.PMID:38360215 | DOI:10.1016/j.jtha.2024.02.003 (Source: Thrombosis and Haemostasis)
Source: Thrombosis and Haemostasis - February 15, 2024 Category: Hematology Authors: X Long Zheng Source Type: research

Acquired von Willebrand syndrome associated with a smoldering multiple myeloma, successfully treated by daratumumab, lenalidomide and dexamethasone
CONCLUSION: Daratumumab, lenalidomide, and dexamethasone demonstrated as a rapid and effective treatment for a patient with severe AvWS and multiple bleeding complications.PMID:38359808 | DOI:10.1159/000536650 (Source: Acta Haematologica)
Source: Acta Haematologica - February 15, 2024 Category: Hematology Authors: Michael Iarossi Marie-Christiane Madeleine Vekemans Nicolas Weynants Cedric Hermans Source Type: research