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Cited by 267Mitochondrial Function and PathologyATP Synthase and ATPases ResearchEnzyme Structure and Function

Assembly of mammalian oxidative phosphorylation complexes I–V and supercomplexes

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Signes, Alba, Fernandez-Vizarra, Erika·2018·Essays in Biochemistry
DOI 10.1042/ebc20170098PMC PMC6056720
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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.

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  • Respiratory complexes consist of 13 subunits encoded by mitochondrial DNA and 78 encoded by nuclear DNA.

    “Respiratory complexes consist of 13 subunits encoded by mitochondrial DNA and 78 encoded by nuclear DNA [80].”

    From Neuronal Apolipoprotein E4 Expression Results in Proteome-Wide Alterations and Compromises Bioenergetic Capacity by Disrupting Mitochondrial Function (2019) · cites this paper as [80]

    The Introduction and the sections on complexes I, III, IV and V describe subunits encoded by mitochondrial and nuclear DNA; their accounts identify seven, one, three and two mitochondrial-encoded subunits, respectively, supporting the total of 13. Our copy does not state a total of 78 nuclear-encoded subunits, so that number is not verified. (LLM, conf=0.82)

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Cited by

Sorted by citations

Authors
Signes, A., & Fernandez-Vizarra, E.
Affiliations
  • University of Cambridge (GB)
  • MRC-Mitochondrial Biology Unit, University of Cambridge, Hills Road, Cambridge CB2 0XY, U.K.
Venue
Essays in Biochemistry
Publisher
Portland Press Ltd.
Published
2018-07-20
Volume
62
Issue
3
Pages
255-270
ISSN
  • 0071-1365 (ISSN-L)
  • 1744-1358
DOI
10.1042/ebc20170098
PMC
PMC6056720
OpenAlex
W2884533811
Cited by
267
References
6
Citations by year
2026: 27 · 2025: 20 · 2024: 40 · 2023: 32 · 2022: 42 · 2021: 50 · 2020: 40 · 2019: 14 · 2018: 1 · 2013: 1
FWCI
2.60
Type
journal article
Language
en
Open access
Licence
Locations
  • Essays in Biochemistry (publishedVersion, cc-by)
  • Europe PMC (PubMed Central) (publishedVersion, cc-by)
  • PubMed Central (publishedVersion, cc-by)
  • PubMed (publishedVersion)
  • Apollo (University of Cambridge) (submittedVersion, cc-by)
  • Padua Research Archive (University of Padova) (submittedVersion)
  • Apollo (cc-by)
Funders
Medical Research Council
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › ATP Synthase and ATPases Research
  • Physical Sciences › Materials Science › Materials Chemistry › Enzyme Structure and Function
Keywords
oxidative phosphorylation, respiratory supercomplexes, respirasome, mitochondrial respiratory chain, mitochondrial disease, assembly factors, molecular chaperones, prosthetic groups, complex I, cytochrome bc1 complex, cytochrome c oxidase, inner mitochondrial membrane
MeSH terms
Animals, Electron Transport, Humans, Mammals, Mitochondria, Multienzyme Complexes, Oxidative Phosphorylation

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