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Cited by 1,028Mitochondrial Function and PathologyAutophagy in Disease and TherapyATP Synthase and ATPases Research

Mitochondrial proteins: from biogenesis to functional networks

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Pfanner, Nikolaus, Warscheid, Bettina, Wiedemann, Nils·2019·Nature Reviews Molecular Cell Biology
DOI 10.1038/s41580-018-0092-0PMC PMC6684368
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Abstract

Mitochondria are essential for the viability of eukaryotic cells as they perform crucial functions in bioenergetics, metabolism and signalling and have been associated with numerous diseases. Recent functional and proteomic studies have revealed the remarkable complexity of mitochondrial protein organization. Protein machineries with diverse functions such as protein translocation, respiration, metabolite transport, protein quality control and the control of membrane architecture interact with each other in dynamic networks. In this Review, we discuss the emerging role of the mitochondrial protein import machinery as a key organizer of these mitochondrial protein networks. The preprotein translocases that reside on the mitochondrial membranes not only function during organelle biogenesis to deliver newly synthesized proteins to their final mitochondrial destination but also cooperate with numerous other mitochondrial protein complexes that perform a wide range of functions. Moreover, these protein networks form membrane contact sites, for example, with the endoplasmic reticulum, that are key for integration of mitochondria with cellular function, and defects in protein import can lead to diseases.

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  • Fungal mitochondrial functions, including cellular respiration, oxidative phosphorylation, ATP synthesis, genetics, metabolic transport, and drug resistance, are highly conserved among different fungal species.

    “Moreover, fungal mitochondrial functions, including cellular respiration, oxidative phosphorylation, ATP synthesis, genetics, metabolic transport, drug resistance, and others, are highly conserved among different fungal species.”

    From Recent Progress in Research on Mitochondrion-Targeted Antifungal Drugs: a Review (2023) · cites this paper as [10]

    The Introduction and “Numerous functions of mitochondria” describe respiration, oxidative phosphorylation, ATP synthesis, a mitochondrial genome, and metabolite transport; page numbers are unavailable in our copy. The paper does not establish that these functions are highly conserved across different fungal species, and the cited passages do not support drug resistance as a mitochondrial function. (LLM, conf=0.86)

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Cited by

Sorted by citations

Authors
Pfanner, N., Warscheid, B., & Wiedemann, N.
Affiliations
University of Freiburg (DE)
Corresponding author
  • Pfanner, Nikolaus
  • Wiedemann, Nils
ORCID
Venue
Nature Reviews Molecular Cell Biology
Publisher
Springer Science and Business Media LLC
Published
2019-01-09
Volume
20
Issue
5
Pages
267-284
ISSN
  • 1471-0072 (ISSN-L)
  • 1471-0080
DOI
10.1038/s41580-018-0092-0
PMC
PMC6684368
OpenAlex
W2909369603
Cited by
1,028
References
13
Citations by year
2026: 126 · 2025: 164 · 2024: 186 · 2023: 124 · 2022: 127 · 2021: 135 · 2020: 119 · 2019: 45 · 2018: 1
FWCI
9.40
Type
journal article
Language
en
Update notices
correction (2021-03-23)
Open access
Licence
Locations
  • Nature Reviews Molecular Cell Biology (publishedVersion)
  • PubMed (publishedVersion)
  • PubMed Central (acceptedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
  • Health Sciences › Medicine › Epidemiology › Autophagy in Disease and Therapy
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › ATP Synthase and ATPases Research
Keywords
mitochondrial proteins, mitochondrial protein import, preprotein translocase, mitochondrial biogenesis, protein translocation, membrane contact sites, endoplasmic reticulum, protein quality control, metabolite transport, mitochondrial respiration, mitochondrial disease
MeSH terms
Animals, Endoplasmic Reticulum, Humans, Mitochondria, Signal Transduction, Protein Transport, Mitochondrial Proteins, Mitochondrial Membranes

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