Blood-induced bone loss in murine hemophilic arthropathy is prevented by blocking the iRhom2/ADAM17/TNF-α pathway

C Haxaire, N Hakobyan, T Pannellini… - Blood, The Journal …, 2018 - ashpublications.org
Hemophilic arthropathy (HA) is a debilitating degenerative joint disease that is a major
manifestation of the bleeding disorder hemophilia A. HA typically begins with hemophilic …

With blood in the joint–what happens next? Could activation of a pro‐inflammatory signalling axis leading to iRhom2/TNFα‐convertase‐dependent release of TNFα …

C Haxaire, CP Blobel - Haemophilia, 2014 - Wiley Online Library
One of the main complications of haemophilia A is haemophilic arthropathy (HA), a
debilitating disease with a significant negative impact on motility and quality of life. Despite …

[HTML][HTML] Hemophilia A and B mice, but not VWF−/−mice, display bone defects in congenital development and remodeling after injury

S Taves, J Sun, EW Livingston, X Chen, J Amiaud… - Scientific reports, 2019 - nature.com
While joint damage is the primary co-morbidity of hemophilia, osteoporosis and osteopenia
are also observed. Coagulation factor VIII deficient (FVIII−/−) mice develop an osteoporotic …

[HTML][HTML] Nuclear factor (NF)‐κB and its associated pathways are major molecular regulators of blood‐induced joint damage in a murine model of hemophilia

D Sen, A Chapla, N Walter, V Daniel… - Journal of Thrombosis …, 2013 - Elsevier
Background The present study was designed to investigate the molecular signaling events
from onset of bleeding through the development of arthropathy in a murine model of …

[HTML][HTML] Maladaptive lymphangiogenesis is associated with synovial iron accumulation and delayed clearance in factor VIII–deficient mice after induced hemarthrosis

EJ Cooke, BC Joseph, CA Nasamran, KM Fisch… - Journal of Thrombosis …, 2023 - Elsevier
Background Mechanisms of iron clearance from hemophilic joints are unknown. Objectives
To better understand mechanisms of iron clearance following joint bleeding in a mouse …

[HTML][HTML] The F8−/− rat as a model of hemophilic arthropathy

KR Sørensen, K Roepstorff, B Wiinberg… - Journal of Thrombosis …, 2016 - Elsevier
Essentials• Validating the F8 rat as a new intermediate‐size animal model of hemophilic
arthropathy.• Factor VIII (FVIII) treated F8−/− rats suffered induced hemarthrosis analyzed by …

[HTML][HTML] Pathophysiological role of synovitis in hemophilic arthropathy development: a two-hit hypothesis

I Calcaterra, G Iannuzzo, F Dell'Aquila… - Frontiers in …, 2020 - frontiersin.org
Despite an increasing access to prophylaxis with clotting factor concentrates, arthropathy
still represents the main chronic complication of hemophilia. Whereas previous studies …

Targeted delivery of miR125a-5p and human Factor VIII attenuates molecular mediators of hemophilic arthropathy

MB Senthilkumar, P Sarangi, S Amit, S Senguttuvan… - Thrombosis …, 2023 - Elsevier
Hemophilic arthropathy (HA) due to repeated bleeding into the joint cavity is a major cause
of morbidity in patients with hemophilia. The molecular mechanisms contributing to this …

Pathophysiology of hemophilic arthropathy and potential targets for therapy

AE Pulles, SC Mastbergen, REG Schutgens… - Pharmacological …, 2017 - Elsevier
Hemophilia is a congenital clotting factor deficiency characterized by spontaneous and
trauma-related bleeding. Spontaneous bleeding shows a predilection for joints, and …

Vascular remodeling underlies rebleeding in hemophilic arthropathy

V Bhat, M Olmer, S Joshi, DL Durden… - American journal of …, 2015 - Wiley Online Library
Hemophilic arthropathy is a debilitating condition that can develop as a consequence of
frequent joint bleeding despite adequate clotting factor replacement. The mechanisms …