Assembly of mammalian oxidative phosphorylation complexes I-V and supercomplexes

Essays Biochem. 2018 Jul 20;62(3):255-270. doi: 10.1042/EBC20170098. Print 2018 Jul 20.

Abstract

The assembly of the five oxidative phosphorylation system (OXPHOS) complexes in the inner mitochondrial membrane is an intricate process. The human enzymes comprise core proteins, performing the catalytic activities, and a large number of 'supernumerary' subunits that play essential roles in assembly, regulation and stability. The correct addition of prosthetic groups as well as chaperoning and incorporation of the structural components require a large number of factors, many of which have been found mutated in cases of mitochondrial disease. Nowadays, the mechanisms of assembly for each of the individual complexes are almost completely understood and the knowledge about the assembly factors involved is constantly increasing. On the other hand, it is now well established that complexes I, III and IV interact with each other, forming the so-called respiratory supercomplexes or 'respirasomes', although the pathways that lead to their formation are still not completely clear. This review is a summary of our current knowledge concerning the assembly of complexes I-V and of the supercomplexes.

Keywords: atp synthase; electron transport chain; mitochondria; oxidative phosphorylation; respiratory chain complex assembly.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Electron Transport
  • Humans
  • Mammals / metabolism
  • Mitochondria / enzymology
  • Mitochondria / metabolism
  • Multienzyme Complexes / metabolism*
  • Oxidative Phosphorylation*

Substances

  • Multienzyme Complexes