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肺动脉高压(pulmonary hypertension，PAH)是临床上常见的一种肺动脉压力增高的病理状态，具有较高的发病率及病死率。研究发现，内皮生长因子、凝血相关因子异常，5-羟色胺，白细胞介素6等炎性因子、低氧诱导因子、转化生长因子β1、结缔组织生长因子(connective tissue growth factor，CTGF)等因子均参与了PAH的发生发展，但具体机制尚不明确。近年来有研究发现，在野百合碱(Monocrotaline，MCT)造模的PAH大鼠肺组织及循环中线粒体偶联因子6(coupling factor 6，CF6)的浓度明显升高，抑制了前列环素(Prostacyclin，PGI2)的活性，提示其可能参与了PAH的发生发展。本文将从CF6的特性、与PGI2的关系，以及与PAH的关系等方面进行讨论。“,”Pulmonary hypertension (PAH) is a common pathological stats disease in clinic, it has a high morbidity and mortality.It was found that abnormal of endothelial growth factor, coagulation-related factors, 5-hydroxytryptamine, interleukin-6 and other inflammatory factors, hypoxia-inducible factors, transforming growth factor-β1 and connective tissue growth factor(CTGF) and so on were involved in the occurrence and development of PAH, but the specific mechanism was still unclear.In recent years, in monocrotaline(MCT) induced PAH rats model, they found that MCT rats had a significant higher concentration of mitochondrial coupling factor 6 (CF6) in both plasma and lung tissue than control group, which inhibited the activity of prostacyclin (PGI2), suggesting that CF6 may be involved in the occurrence and development of PAH.This paper will discuss the characteristics of CF6, the relationship between PGI2 and CF6, the relationship between PAH and CF6.