P. Marambaud

CDK6-mediated endothelial cell cycle acceleration drives arteriovenous malformations in hereditary hemorrhagic telangiectasia

This study identifies CDK6-driven cell cycle acceleration in endothelial cells as a driver of arteriovenous malformations in HHT, suggesting CDK4/6 inhibitors as potential therapeutic options.

Oxidative stress-induced MMP- and γ-secretase-dependent VE-cadherin processing is modulated by the proteasome and BMP9/10

This study shows oxidative stress induces VE-cadherin proteolysis via MMPs and γ-secretase in HUVECs, with BMP9/10 reducing this effect and protecting against apoptosis.

Limb Specific Failure of Proliferation and Translation in the Mesenchyme Leads to Skeletal Defects in Diamond Blackfan Anemia

This study identifies site-specific skeletal defects in DBA by showing limb-specific mesenchymal stem cell proliferation and translation failures linked to WNT signaling and p53.

Sirolimus plus nintedanib treats vascular pathology in HHT mouse models

This study shows that combining sirolimus and nintedanib treats vascular abnormalities in HHT mouse models by targeting multiple pathways.

Tacrolimus Rescues Endothelial ALK1 Loss-Of-Function Signaling And Improves HHT Vascular Pathology

This study identifies tacrolimus as a drug that activates Smad1/5/8 signaling and reduces vascular pathology in HHT models.

A mouse model of hereditary hemorrhagic telangiectasia generated by transmammary-delivered immunoblocking of BMP9 and BMP10

This study uses transmammary delivery of anti-BMP9/10 antibodies to induce HHT-like vascular defects in mouse neonates, revealing consistent retinal pathology and increased angiopoietin-2 expression.