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Background Pulmonary hypertension (PH) is a life-threatening disease. A pro-inflammatory milieu drives maladaptive right ventricular (RV) remodelling and failure. There is an unmet need for RV-targeted pharmaco-therapies. We investigated the effect of P2X7 receptor (P2X7R) inhibition on the pulmonary vasculature and RV remodelling using the novel P2X7R antagonist, PKT100. Methods C57BL/6 mice were administered intratracheal bleomycin or saline and treated with PKT100 (0.2 mg/kg/day) or DMSO-vehicle. RV was assessed by right heart cathet