Cited by 71Mitochondrial Function and PathologyATP Synthase and ATPases ResearchAutophagy in Disease and Therapy

Coupling of import and assembly pathways in mitochondrial protein biogenesis

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Grevel, Alexander, Pfanner, Nikolaus, Becker, Thomas·2019·Biological Chemistry

Abstract

Biogenesis and function of mitochondria depend on the import of about 1000 precursor proteins that are produced on cytosolic ribosomes. The translocase of the outer membrane (TOM) forms the entry gate for most proteins. After passage through the TOM channel, dedicated preprotein translocases sort the precursor proteins into the mitochondrial subcompartments. Many proteins have to be assembled into oligomeric membrane-integrated complexes in order to perform their functions. In this review, we discuss a dual role of mitochondrial preprotein translocases in protein translocation and oligomeric assembly, focusing on the biogenesis of the TOM complex and the respiratory chain. The sorting and assembly machinery (SAM) of the outer mitochondrial membrane forms a dynamic platform for coupling transport and assembly of TOM subunits. The biogenesis of the cytochrome c oxidase of the inner membrane involves a molecular circuit to adjust translation of mitochondrial-encoded core subunits to the availability of nuclear-encoded partner proteins. Thus, mitochondrial protein translocases not only import precursor proteins but can also support their assembly into functional complexes.

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  • Incorporation of mitochondrial protein precursors into specific subcompartments requires specific and highly regulated import machinery.

    “The incorporation of these precursors into specific mitochondria subcompartments such as the outer mitochondrial membrane (OMM), the intermembrane space (IMS), the inner mitochondrial membrane (IMM) and the mitochondrial matrix requires specific and highly regulated import machinery [35].”

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Authors
Grevel, A., Pfanner, N., & Becker, T.
Affiliations
University of Freiburg (DE)
Corresponding author
  • Pfanner, Nikolaus
  • Becker, Thomas
Venue
Biological Chemistry
Published
2019-09-11
Volume
401
Issue
1
Pages
117-129
ISSN
  • 1431-6730 (ISSN-L)
  • 1437-4315
OpenAlex
W2972571572
Cited by
71
References
141
Citations by year
2026: 3 · 2025: 5 · 2024: 8 · 2023: 20 · 2022: 6 · 2021: 14 · 2020: 14 · 2019: 1
FWCI
0.77
Type
journal article
Language
en
Open access
Locations
  • Biological Chemistry (publishedVersion)
  • PubMed (publishedVersion)
Funders
Deutsche Forschungsgemeinschaft (PF 202/9‐1, 202/9-1, EXC‐2189, BE 4679 / 2‐2, 390939984)
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
  • Health Sciences › Medicine › Epidemiology › Autophagy in Disease and Therapy
Keywords
mitochondrial protein biogenesis, TOM complex, preprotein translocase, mitochondrial protein import, protein assembly, cytochrome c oxidase, respiratory chain assembly, translational regulation, mitochondrial outer membrane, inner membrane
MeSH terms
Mitochondrial Precursor Protein Import Complex Proteins, Carrier Proteins, Mitochondria, Ribosomes, Saccharomyces cerevisiae, Protein Biosynthesis, Protein Transport, Mitochondrial Proteins, Mitochondrial Membrane Transport Proteins, Mitochondrial Membranes

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