Laboratory of Systems and Synthetic Biology, Chair of Drug and Cosmetics Biotechnology, Faculty of Chemistry, Warsaw University of Technology, Noakowskiego 3, 00-664 Warsaw, Poland.
School of Medicine, V. N. Karazin Kharkiv National University, 61022 Kharkiv, Ukraine.
Int J Mol Sci. 2024 Sep 15;25(18):9950. doi: 10.3390/ijms25189950.
This survey reviews modern ideas on the structure and functions of mitochondrial and cytosolic aconitase isoenzymes in eukaryotes. Cumulative experimental evidence about mitochondrial aconitases (Aco2) as one of the main targets of reactive oxygen and nitrogen species is generalized. The important role of Aco2 in maintenance of homeostasis of the intracellular iron pool and maintenance of the mitochondrial DNA is discussed. The role of Aco2 in the pathogenesis of some neurodegenerative diseases is highlighted. Inactivation or dysfunction of Aco2 as well as mutations found in the ACO2 gene appear to be significant factors in the development and promotion of various types of neurodegenerative diseases. A restoration of efficient mitochondrial functioning as a source of energy for the cell by targeting Aco2 seems to be one of the promising therapeutic directions to minimize progressive neurodegenerative disorders.
本综述回顾了真核生物中线粒体和细胞质 aconitase 同工酶的结构和功能的现代观点。总结了关于线粒体 aconitase（Aco2）作为活性氧和氮物种的主要靶标之一的累积实验证据。讨论了 Aco2 在维持细胞内铁池的内稳态和维持线粒体 DNA 方面的重要作用。强调了 Aco2 在一些神经退行性疾病发病机制中的作用。Aco2 的失活或功能障碍以及在 ACO2 基因中发现的突变似乎是各种类型的神经退行性疾病发展和促进的重要因素。通过靶向 Aco2 恢复作为细胞能量来源的有效的线粒体功能似乎是最小化进行性神经退行性疾病的有前途的治疗方向之一。
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