Australian Centre for Blood Diseases, Central Clinical School, Monash University and Alfred Health, Monash AMREP Building, Level 1, Walkway, via The Alfred Centre, 99 Commercial Road, Melbourne, VIC, 3004, Australia.
Purinergic Signal. 2022 Dec;18(4):409-419. doi: 10.1007/s11302-022-09889-9. Epub 2022 Aug 10.
Pulmonary arterial hypertension (PAH) is a devastating progressive disease characterised by pulmonary arterial vasoconstriction and vascular remodelling. Endothelial dysfunction has emerged as a contributing factor in the development of PAH. However, despite progress in the understanding of the pathophysiology of this disease, current therapies fail to impact upon long-term outcomes which remain poor in most patients. Recent observations have suggested the disturbances in the balance between ATP and adenosine may be integral to the vascular remodelling seen in PAH. CD39 is an enzyme important in regulating these nucleos(t)ides which may also provide a novel pathway to target for future therapies. This review summarises the role of adenosine signalling in the development and progression of PAH and highlights the therapeutic potential of CD39 for treatment of PAH.
肺动脉高压（PAH）是一种破坏性的进行性疾病，其特征为肺动脉收缩和血管重塑。内皮功能障碍已成为 PAH 发展的一个促成因素。然而，尽管对该疾病病理生理学的理解取得了进展，但目前的治疗方法仍无法影响大多数患者的长期预后，预后仍然较差。最近的观察结果表明，ATP 和腺苷之间平衡的紊乱可能是 PAH 中所见血管重塑的一个组成部分。CD39 是一种在调节这些核苷酸方面很重要的酶，它也可能为未来的治疗提供一个新的靶点。本综述总结了腺苷信号在 PAH 的发生和进展中的作用，并强调了 CD39 在治疗 PAH 方面的治疗潜力。
