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 …
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 …
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 …
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
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 …
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
Background Mechanisms of iron clearance from hemophilic joints are unknown. Objectives
To better understand mechanisms of iron clearance following joint bleeding in a mouse …
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 …
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 …
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 …
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 …
trauma-related bleeding. Spontaneous bleeding shows a predilection for joints, and …
Vascular remodeling underlies rebleeding in hemophilic arthropathy
Hemophilic arthropathy is a debilitating condition that can develop as a consequence of
frequent joint bleeding despite adequate clotting factor replacement. The mechanisms …
frequent joint bleeding despite adequate clotting factor replacement. The mechanisms …