Courboulin Audrey, Ranchoux Benoît, Cohen-Kaminsky Sylvia, Perros Frédéric, Bonnet Sébastien
aInserm UMR-S 999, Faculté de Médecine, Hôpital Marie Lannelongue, Université Paris-Sud, Université Paris Saclay, Le Plessis Robinson, France bPulmonary Hypertension Research Group, Centre de Recherche de l'Institut Universitaire de Cardiologie et de Pneumologie de Québec, Université Laval, Québec, Canada *Audrey Courboulin and Benoît Ranchoux contributed equally to the writing of this article.
Curr Opin Oncol. 2016 Jan;28(1):72-82. doi: 10.1097/CCO.0000000000000253.
Pulmonary arterial hypertension (PAH) is a rare disease with poor prognosis and no therapeutics. PAH is characterized by severe remodeling of precapillary pulmonary arteries, leading to increased vascular resistance, pulmonary hypertension compensatory right ventricular hypertrophy, then heart failure and death. PAH pathogenesis shares similarities with carcinogenesis such as excessive cell proliferation, apoptosis resistance, metabolic shifts, or phenotypic transition. Although PAH is not a cancer, comparison of analogous mechanisms between PAH and cancer led to the concept of a cancer-like disease to emerge. MicroRNAs (miRNAs) are small noncoding RNAs involved in the regulation of posttranscriptional gene expression. miRNA dysregulations have been reported as promoter of the development of various diseases including cancers.
Recent studies revealed that miRNA dysregulations also occur in PAH pathogenesis. In PAH, different miRNAs have been implicated to be the main features of PAH pathophysiology (in pulmonary inflammation, vascular remodeling, angiogenesis, and right heart hypertrophy).
The review summarizes the implication of miRNA dysregulation in PAH development and discusses the similarities and differences with those observed in cancers.
肺动脉高压（PAH）是一种预后不良且尚无有效治疗方法的罕见疾病。PAH的特征是肺前毛细血管严重重塑，导致血管阻力增加、肺动脉高压、代偿性右心室肥厚，进而发展为心力衰竭和死亡。PAH的发病机制与癌症发生有相似之处，如细胞过度增殖、抗凋亡、代谢改变或表型转变。虽然PAH不是癌症，但对PAH和癌症之间类似机制的比较催生了类癌疾病的概念。微小RNA（miRNA）是参与转录后基因表达调控的小非编码RNA。据报道，miRNA失调是包括癌症在内的各种疾病发展的促进因素。
最近的研究表明，miRNA失调也发生在PAH的发病机制中。在PAH中，不同的miRNA被认为是PAH病理生理学（肺部炎症、血管重塑、血管生成和右心肥厚）的主要特征。
本综述总结了miRNA失调在PAH发展中的作用，并讨论了其与癌症中观察到的情况的异同。
