BMB Reports 2018; 51(11): 549-556
Roles of mitochondria in neuronal development
Geurim Son1 & Jinju Han1,2,*
1Biomedical Science and Engineering Interdisciplinary Program, Korea Advanced Institute of Science and Technology (KAIST), Daejeon 34141, Korea, 2Graduate School of Medical Science and Engineering, KAIST, Daejeon 34141, Korea
Correspondence to: Tel: +82-42-350-4862; Fax: +82-42-350-4240; E-mail:
Received: September 4, 2018; Published online: November 30, 2018.
© Korean Society for Biochemistry and Molecular Biology. All rights reserved.

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Mitochondria are ubiquitous and multi-functional organelles involved in diverse metabolic processes, namely energy production and biomolecule synthesis. The intracellular mitochondrial morphology and distribution change dynamically, which reflect the metabolic state of a given cell type. A dramatic change of the mitochondrial dynamics has been observed in early development that led to further investigations on the relationship between mitochondria and the process of development. A significant developmental process to focus on, in this review, is a differentiation of neural progenitor cells into neurons. Information on how mitochondria-regulated cellular energetics is linked to neuronal development will be discussed, followed by functions of mitochondria and associated diseases in neuronal development. Lastly, the potential use of mitochondrial features in analyzing various neurodevelopmental diseases will be addressed.
Keywords: Energy metabolism, Mitochondria, Neural stem cell, Neurodevelopmental diseases, Neuronal development
Fig. 1. Mitochondrial proteins, functions and dynamics. (A) Major function of mitochondria is energy production through OXPHOS. Glycolysis occurring in the cytosol produce pyruvate, which is necessary to fuel the tricarboxylic acid (TCA) cycle. The pentose phosphate pathway (PPP) is a shunt for glycolysis. Through the PPP, cells acquire required components for other cellular processes including nucleotide synthesis. In mitochondria, beta-oxidation occurs as the other mechanism of converting lipid to generate energy. (B) Most proteins localized in the mitochondria are produced from the nuclear genome (nDNA) and transported into mitochondria. Mitochondria contain its own genome (mitochondrial DNA, mtDNA) and produce 13 proteins comprising oxidative phosphorylation (OXPHOS) complex. (C) Dynamically changing morphology of mitochondria through continuous cycle of fusion and fission.

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