Institute of Physiology, Justus-Liebig University, 35392 Giessen, Germany.
Int J Mol Sci. 2021 Jan 20;22(3):987. doi: 10.3390/ijms22030987.
In the heart, connexins form gap junctions, hemichannels, and are also present within mitochondria, with connexin 43 (Cx43) being the most prominent connexin in the ventricles. Whereas the role of Cx43 is well established for the healthy and diseased left ventricle, less is known about the importance of Cx43 for the development of right ventricular (RV) dysfunction. The present article focusses on the importance of Cx43 for the developing heart. Furthermore, we discuss the expression and localization of Cx43 in the diseased RV, i.e., in the tetralogy of Fallot and in pulmonary hypertension, in which the RV is affected, and RV hypertrophy and failure occur. We will also introduce other Cx molecules that are expressed in RV and surrounding tissues and have been reported to be involved in RV pathophysiology. Finally, we highlight therapeutic strategies aiming to improve RV function in pulmonary hypertension that are associated with alterations of Cx43 expression and function.
在心脏中，连接蛋白形成间隙连接、半通道，并且也存在于线粒体中，其中连接蛋白 43（Cx43）是心室中最突出的连接蛋白。虽然 Cx43 对于健康和患病的左心室的作用已经得到很好的确立，但对于 Cx43 对于右心室（RV）功能障碍的发展的重要性知之甚少。本文重点介绍了 Cx43 对于发育中的心脏的重要性。此外，我们还讨论了 Cx43 在患病的 RV 中的表达和定位，即在法洛四联症和肺动脉高压中，RV 受到影响，并且发生 RV 肥大和衰竭。我们还将介绍在 RV 和周围组织中表达的其他 Cx 分子，这些分子已被报道与 RV 病理生理学有关。最后，我们强调了旨在改善肺动脉高压中 RV 功能的治疗策略，这些策略与 Cx43 表达和功能的改变有关。
