von Willebrand factor biosynthesis, secretion, and clearance: connecting the far ends

PJ Lenting, OD Christophe… - Blood, The Journal of the …, 2015 - ashpublications.org
To understand the placement of a certain protein in a physiological system and the
pathogenesis of related disorders, it is not only of interest to determine its function but also …

Phenotypic heterogeneity of the endothelium: II. Representative vascular beds

WC Aird - Circulation research, 2007 - Am Heart Assoc
Endothelial cells, which form the inner cellular lining of blood vessels and lymphatics,
display remarkable heterogeneity in structure and function. This is the second of a 2-part …

Life in the shadow of a dominant partner: the FVIII-VWF association and its clinical implications for hemophilia A

SW Pipe, RR Montgomery, KP Pratt… - Blood, The Journal …, 2016 - ashpublications.org
A normal hemostatic response to vascular injury requires both factor VIII (FVIII) and von
Willebrand factor (VWF). In plasma, VWF and FVIII normally circulate as a noncovalent …

Functional architecture of Weibel-Palade bodies

KM Valentijn, JE Sadler, JA Valentijn… - Blood, The Journal …, 2011 - ashpublications.org
Abstract Weibel-Palade bodies (WPBs) are elongated secretory organelles specific to
endothelial cells that contain von Willebrand factor (VWF) and a variety of other proteins that …

Murine coagulation factor VIII is synthesized in endothelial cells

LA Everett, ACA Cleuren, RN Khoriaty… - Blood, The Journal of …, 2014 - ashpublications.org
The primary cellular source of factor VIII (FVIII) biosynthesis is controversial, with
contradictory evidence supporting an endothelial or hepatocyte origin. LMAN1 is a cargo …

A conditional knockout mouse model reveals endothelial cells as the principal and possibly exclusive source of plasma factor VIII

SA Fahs, MT Hille, Q Shi, H Weiler… - Blood, The Journal of …, 2014 - ashpublications.org
The cellular source of coagulation factor VIII (FVIII) remains controversial. Like many
coagulation proteins, FVIII is produced in the liver, and FVIII synthesis has long been …

[HTML][HTML] Preliminary post-mortem COVID-19 evidence of endothelial injury and factor VIII hyperexpression

L Cipolloni, F Sessa, G Bertozzi, B Baldari, S Cantatore… - Diagnostics, 2020 - mdpi.com
(1) Background: The current outbreak of COVID-19 infection is an ongoing challenge and a
major threat to public health that requires surveillance, prompt diagnosis, as well as …

Factor VIII and von Willebrand factor interaction: biological, clinical and therapeutic importance

V Terraube, JS O'donnell, PV Jenkins - Haemophilia, 2010 - Wiley Online Library
The interaction of factor VIII (FVIII) with von Willebrand Factor (VWF) is of direct clinical
significance in the diagnosis and treatment of patients with haemophilia A and von …

[HTML][HTML] Therapeutic mesenchymal stromal cells for immunotherapy and for gene and drug delivery

G Almeida-Porada, AJ Atala, CD Porada - Molecular Therapy-Methods & …, 2020 - cell.com
Mesenchymal stromal cells (MSCs) possess several fairly unique properties that, when
combined, make them ideally suited for cellular-based immunotherapy and as vehicles for …

von Willebrand factor to the rescue

SF De Meyer, H Deckmyn… - Blood, The Journal of …, 2009 - ashpublications.org
Abstract von Willebrand factor (VWF) is a large multimeric adhesive glycoprotein with
complex roles in thrombosis and hemostasis. Abnormalities in VWF give rise to a variety of …