SpringerProtocols: Abstract: Oxidative Phosphorylation: Synthesis of Mitochondrially Encoded Proteins and Assembly of Individual Structural Subunits into Functional Holoenzyme Complexes

Oxidative Phosphorylation: Synthesis of Mitochondrially Encoded Proteins and Assembly of Individual Structural Subunits into Functional Holoenzyme Complexes

By: Scot C. Leary², Florin Sasarman²

Abstract

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The bulk of ATP consumed by various cellular processes in higher eukaryotes is normally produced by five multimeric protein complexes (I–V) embedded within the inner mitochondrial membrane, in a process known as oxidative phosphorylation (OXPHOS). Maintenance of energy homeostasis under most physiological conditions is therefore contingent upon the ability of OXPHOS to meet cellular changes in bioenergetic demand, with a chronic failure to do so being a frequent cause of human disease. With the exception of Complex II, the structural subunits of OXPHOS complexes are encoded by both the nuclear and the mitochondrial genomes. The physical separation of the two genomes necessitates that the expression of the 13 mitochondrially encoded polypeptides be co-ordinated with that of relevant nuclear-encoded partners in order to assemble functional holoenzyme complexes. Complex biogenesis is a highly ordered process, and several nuclear-encoded factors that function at distinct stages in the assembly of individual OXPHOS complexes have been identified.

Affiliation(s): (2) Montreal Neurological Institute and Department of Human Genetics, McGill University, Montreal, QC, Canada

Book Title: Mitochondrial DNA: Methods and Protocols

Series: Methods in Molecular Biology | Volume: 554 | Pub. Date: Sep-01-2009 | Page Range: 143-162 | DOI: 10.1007/978-1-59745-521-3_10

Subject: Cell Biology

Key Words: Mitochondria - oxidative phosphorylation mtDNA - pulse-chase labeling - mitochondrial translation - holoenzyme assembly - blue native PAGE

References

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