Department of General Pediatrics, Neonatology and Pediatric Cardiology, University Children's Hospital, Heinrich Heine University, Düsseldorf, Germany.
Department of General Pediatrics, Neonatology and Pediatric Cardiology, University Children's Hospital, Heinrich Heine University, Düsseldorf, Germany; Max Delbrück Center for Molecular Medicine (MDC), Berlin, Germany.
Int J Biochem Cell Biol. 2020 Jun;123:105760. doi: 10.1016/j.biocel.2020.105760. Epub 2020 Apr 25.
Mitochondria exhibit high degree of heterogeneity within various tissues, including differences in terms of morphology, quantity, or function. Mitochondria can even vary among distinct sub-compartments of the same cell. Emerging evidence suggest that the molecular diversity of mitochondria can influence the identity and functionality of a given cell type. Human pathologies affecting mitochondria typically cause tissue and cell-type-specific impairment. Mitochondrial diversity could thus play a contributing role not only in physiological cell fate specification but also during pathological disease development. In this review, we discuss the role of mitochondrial diversity in brain function during health and disease. Recent advances in induced pluripotent stem cells (iPSCs) research and the derivation of cerebral organoids could provide novel opportunities to unveil the role of mitochondrial heterogeneity for the function of the human brain. Mitochondrial diversity might be at the bases of the cell-type-specific vulnerability of mitochondrial disorders and may represent an underappreciated target of disease intervention.
线粒体在各种组织中表现出高度的异质性，包括形态、数量或功能上的差异。线粒体甚至在同一细胞的不同亚区室之间也可能有所不同。新出现的证据表明，线粒体的分子多样性可以影响特定细胞类型的身份和功能。影响线粒体的人类病理通常会导致组织和细胞类型特异性损伤。因此，线粒体多样性不仅可以在生理细胞命运特化中发挥作用，而且在病理性疾病发展中也可以发挥作用。在这篇综述中，我们讨论了线粒体多样性在健康和疾病状态下对大脑功能的作用。诱导多能干细胞（iPSC）研究和大脑类器官的衍生方面的最新进展，为揭示线粒体异质性对人类大脑功能的作用提供了新的机会。线粒体多样性可能是线粒体疾病细胞类型特异性易损性的基础，也可能是疾病干预的一个未被充分认识的靶点。
