ELKH-SZTE Neuroscience Research Group, Danube Neuroscience Research Laboratory, Eötvös Loránd Research Network, University of Szeged (ELKH-SZTE), Tisza Lajos krt. 113, H-6725 Szeged, Hungary.
Department of Neurology, Albert Szent-Györgyi Medical School, University of Szeged, Semmelweis u. 6, H-6725 Szeged, Hungary.
Cells. 2022 Aug 21;11(16):2607. doi: 10.3390/cells11162607.
Nearly half a century has passed since the discovery of cytoplasmic inheritance of human chloramphenicol resistance. The inheritance was then revealed to take place maternally by mitochondrial DNA (mtDNA). Later, a number of mutations in mtDNA were identified as a cause of severe inheritable metabolic diseases with neurological manifestation, and the impairment of mitochondrial functions has been probed in the pathogenesis of a wide range of illnesses including neurodegenerative diseases. Recently, a growing number of preclinical studies have revealed that animal behaviors are influenced by the impairment of mitochondrial functions and possibly by the loss of mitochondrial stress resilience. Indeed, as high as 54% of patients with one of the most common primary mitochondrial diseases, mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome, present psychiatric symptoms including cognitive impairment, mood disorder, anxiety, and psychosis. Mitochondria are multifunctional organelles which produce cellular energy and play a major role in other cellular functions including homeostasis, cellular signaling, and gene expression, among others. Mitochondrial functions are observed to be compromised and to become less resilient under continuous stress. Meanwhile, stress and inflammation have been linked to the activation of the tryptophan (Trp)-kynurenine (KYN) metabolic system, which observably contributes to the development of pathological conditions including neurological and psychiatric disorders. This review discusses the functions of mitochondria and the Trp-KYN system, the interaction of the Trp-KYN system with mitochondria, and the current understanding of the involvement of mitochondria and the Trp-KYN system in preclinical and clinical studies of major neurological and psychiatric diseases.
自发现人类氯霉素抗性的细胞质遗传以来,已经过去了将近半个世纪。当时发现这种遗传是通过线粒体 DNA(mtDNA)的母系遗传发生的。后来,发现了 mtDNA 中的许多突变是导致严重遗传性代谢疾病和神经表现的原因,并且在包括神经退行性疾病在内的广泛疾病的发病机制中探究了线粒体功能的损害。最近,越来越多的临床前研究表明,动物的行为受到线粒体功能损害的影响,并且可能受到线粒体应激弹性丧失的影响。事实上,高达 54%的最常见的原发性线粒体疾病之一,即线粒体脑肌病伴高乳酸血症和卒中样发作(MELAS)综合征的患者,表现出包括认知障碍、情绪障碍、焦虑和精神病在内的精神症状。线粒体是多功能细胞器,产生细胞能量,并在其他细胞功能(包括内稳态、细胞信号传导和基因表达等)中发挥主要作用。在持续的压力下,线粒体功能被观察到受损且变得不那么有弹性。同时,应激和炎症与色氨酸(Trp)-犬尿氨酸(KYN)代谢系统的激活有关,该系统明显有助于包括神经和精神障碍在内的病理状况的发展。这篇综述讨论了线粒体和 Trp-KYN 系统的功能、Trp-KYN 系统与线粒体的相互作用,以及线粒体和 Trp-KYN 系统在主要神经和精神疾病的临床前和临床研究中的参与的当前理解。