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Oxidative Phosphorylation in Health and Disease pp 28–42Cite as

Molecular Biology of the OXPHOS System

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Part of the book series: Medical Intelligence Unit ((MIUN))

Abstract

The mitochondrion contains a circular DNA genome (mtDNA) that serves as the basis for its own genetic system. This system is semiautonomous because the coding capacity of mtDNA is limited to 13 subunits of the respiratory chain apparatus and the rRNAs and tRNAs necessary for their translation. The inheritance of mtDNA differs from that of nuclear DNA in that it segregates randomly during mitosis and meiosis and is transmitted exclusively through the female germ line. Nucleus-encoded enzymes and factors direct the transcription and replication of mtDNA within the mitochondrial matrix. Mitochondrial translation also relies upon nucleus-encoded ribosomal proteins, synthetases and translation factors. In recent years, molecular mechanisms for the bi-genomic control of mitochondrial biogenesis and function have been elucidated.

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  1. Cell and Molecular Biology, Northwestern Medical School, Chicago, Illinois, USA

    Richard C. Scarpulla

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Scarpulla, R.C. (2004). Molecular Biology of the OXPHOS System. In: Oxidative Phosphorylation in Health and Disease. Medical Intelligence Unit. Springer, Boston, MA. https://doi.org/10.1007/0-387-26992-4_2

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