ATP5PF and pulmonary arterial hypertension: Based on the model, we demonstrated that (1) CF6 was significantly upregulated in lungs and serum of PAH rats, specifically, in the lung tissues; (2) CF6 gene silencing using AAV2-CF6-shRNA induced down-regulation of CF6 protein in the lung tissues of the PAH rats, along with reversed prostacyclin synthesis; and (3) transfer of CF6 shRNA ameliorated MCT-induced pulmonary vascular remodeling and PAH progression.