Emerging roles of protein mannosylation in inflammation and infection

I Loke, D Kolarich, NH Packer… - Molecular aspects of …, 2016 - Elsevier
Proteins are frequently modified by complex carbohydrates (glycans) that play central roles
in maintaining the structural and functional integrity of cells and tissues in humans and lower …

[HTML][HTML] The contribution of the sinusoidal endothelial cell receptors CLEC4M, stabilin-2, and SCARA5 to VWF–FVIII clearance in thrombosis and hemostasis

LL Swystun, A Michels, D Lillicrap - Journal of Thrombosis and …, 2023 - Elsevier
Quantitative abnormalities in factor VIII (FVIII) and its binding partner, von Willebrand factor
(VWF), are associated with an increased risk of bleeding or thrombosis, and pathways that …

[HTML][HTML] The endothelial cell receptor stabilin-2 regulates VWF-FVIII complex half-life and immunogenicity

LL Swystun, JD Lai, C Notley… - The Journal of …, 2018 - Am Soc Clin Investig
Quantitative abnormalities of the von Willebrand factor–factor VIII (VWF-FVIII) complex
associate with inherited bleeding or thrombotic disorders. Receptor-mediated interactions …

Biological considerations of plasma-derived and recombinant factor VIII immunogenicity

J Lai, C Hough, J Tarrant… - Blood, The Journal of the …, 2017 - ashpublications.org
In hemophilia A, the most severe complication of factor VIII (FVIII) replacement therapy
involves the formation of FVIII neutralizing antibodies, also known as inhibitors, in 25% to …

[HTML][HTML] Circumventing furin enhances factor VIII biological activity and ameliorates bleeding phenotypes in hemophilia models

JI Siner, BJ Samelson-Jones, JM Crudele, RA French… - JCI insight, 2016 - ncbi.nlm.nih.gov
Processing by the proprotein convertase furin is believed to be critical for the biological
activity of multiple proteins involved in hemostasis, including coagulation factor VIII (FVIII) …

To serve and protect: the modulatory role of von Willebrand factor on factor VIII immunogenicity

RB Hartholt, AS van Velzen, I Peyron, A Ten Brinke… - Blood reviews, 2017 - Elsevier
Hemophilia A is a bleeding disorder characterized by the absence or dysfunction of blood
coagulation factor VIII (FVIII). Patients are treated with regular infusions of FVIII concentrate …

Venous thromboembolism, factor VIII and chronic kidney disease

KL Cheung, BA Bouchard, M Cushman - Thrombosis research, 2018 - Elsevier
Chronic kidney disease (CKD) affects 30 million Americans and is associated with
approximately a two-fold increased risk of venous thromboembolism (VTE). There is a …

Inhibitor formation in congenital hemophilia A: an immunological perspective

S Delignat, J Rayes, J Russick… - … in Thrombosis and …, 2018 - thieme-connect.com
The immunogenicity of therapeutic factor VIII (FVIII) in patients with hemophilia A has been
puzzling scientific and clinical communities for more than 3 decades. Indeed, the …

Inhibitors in nonsevere hemophilia a: what is known and searching for the unknown

A Abdi, S Linari, L Pieri, J Voorberg… - … in thrombosis and …, 2018 - thieme-connect.com
Nonsevere hemophilia A (NSHA) is an inherited X-linked bleeding disorder, caused by
mutations of the F8 gene, leading to decreases of clotting factor VIII (FVIII) levels to 1 to 40 …

[HTML][HTML] Factor VIII: Perspectives on immunogenicity and tolerogenic strategies for Hemophilia A patients

S Khalilian, M Motovali-Bashi… - International Journal of …, 2020 - ncbi.nlm.nih.gov
A major complication in treating hemophilia A is the development of neutralizing antibodies
(inhibitors) against therapeutic administered factor VIII (FVIII), which occurs in approximately …