Atlante Anna, Valenti Daniela
Institute of Biomembranes, Bioenergetics and Molecular Biotechnologies (IBIOM), National Research Council (CNR), Via G. Amendola 122/O, 70126 Bari, Italy.
Curr Issues Mol Biol. 2023 May 19;45(5):4451-4479. doi: 10.3390/cimb45050283.
Mitochondria have made a long evolutionary path from ancient bacteria immigrants within the eukaryotic cell to become key players for the cell, assuming crucial multitasking skills critical for human health and disease. Traditionally identified as the powerhouses of eukaryotic cells due to their central role in energy metabolism, these chemiosmotic machines that synthesize ATP are known as the only maternally inherited organelles with their own genome, where mutations can cause diseases, opening up the field of mitochondrial medicine. More recently, the omics era has highlighted mitochondria as biosynthetic and signaling organelles influencing the behaviors of cells and organisms, making mitochondria the most studied organelles in the biomedical sciences. In this review, we will especially focus on certain 'novelties' in mitochondrial biology "left in the shadows" because, although they have been discovered for some time, they are still not taken with due consideration. We will focus on certain particularities of these organelles, for example, those relating to their metabolism and energy efficiency. In particular, some of their functions that reflect the type of cell in which they reside will be critically discussed, for example, the role of some carriers that are strictly functional to the typical metabolism of the cell or to the tissue specialization. Furthermore, some diseases in whose pathogenesis, surprisingly, mitochondria are involved will be mentioned.
线粒体经历了漫长的进化历程,从真核细胞内古老的细菌入侵者,发展成为细胞的关键角色,具备了对人类健康和疾病至关重要的关键多任务技能。由于其在能量代谢中的核心作用,传统上线粒体被视为真核细胞的动力源,这些合成ATP的化学渗透机器是已知唯一具有自身基因组的母系遗传细胞器,其突变可导致疾病,从而开启了线粒体医学领域。最近,组学时代突出了线粒体作为影响细胞和生物体行为的生物合成和信号细胞器的作用,使线粒体成为生物医学科学中研究最多的细胞器。在这篇综述中,我们将特别关注线粒体生物学中某些“被忽视的新发现”,因为尽管它们已被发现一段时间,但仍未得到应有的重视。我们将关注这些细胞器的某些特性,例如与它们的代谢和能量效率相关的特性。特别是,将批判性地讨论它们的一些反映其所在细胞类型的功能,例如一些对细胞典型代谢或组织特化严格起作用的载体的作用。此外,还将提及一些令人惊讶的线粒体参与其发病机制的疾病。