Mitochondrial protein import clogging as a mechanism of disease Article Swipe
Liam P. Coyne
,
Xiaowen Wang
,
Jiyao Song
,
Ebbing P. de Jong
,
Karin Schneider
,
Paul T. Massa
,
Frank A. Middleton
,
Thomas Becker
,
Xin Jie Chen
·
YOU?
·
· 2022
· Open Access
·
· DOI: https://doi.org/10.1101/2022.09.20.508789
YOU?
·
· 2022
· Open Access
·
· DOI: https://doi.org/10.1101/2022.09.20.508789
Mitochondrial biogenesis requires the import of >1,000 mitochondrial preproteins from the cytosol. Most studies on mitochondrial protein import are focused on the core import machinery. Whether and how the biophysical properties of substrate preproteins affect overall import efficiency is underexplored. Here, we show that protein traffic into mitochondria is disrupted by amino acid substitutions in a single substrate preprotein. Pathogenic missense mutations in adenine nucleotide translocase 1 (Ant1), and its yeast ortholog Aac2, cause the protein to accumulate along the protein import pathway, thereby obstructing general protein translocation into mitochondria. This impairs mitochondrial respiration, cytosolic proteostasis and cell viability independent of Ant1’s nucleotide transport activity. The mutations act synergistically, as double mutant Aac2/Ant1 cause severe clogging primarily at the Translocase of the Outer Membrane (TOM) complex. This confers extreme toxicity in yeast. In mice, expression of a super-clogger Ant1 variant led to an age-dependent dominant myopathy that phenocopies Ant1-induced human disease, suggesting clogging as a mechanism of disease. We propose that secondary structures of mitochondrial preproteins play an essential role in preventing clogging and disease.
Related Topics
Concepts
Biology
Proteostasis
Mitochondrion
Cell biology
Intermembrane space
Translocase
Transport protein
ATP–ADP translocase
Mitochondrial biogenesis
Inner mitochondrial membrane
Biochemistry
Bacterial outer membrane
Chromosomal translocation
Gene
Escherichia coli
Metadata
- Type
- preprint
- Language
- en
- Landing Page
- https://doi.org/10.1101/2022.09.20.508789
- https://www.biorxiv.org/content/biorxiv/early/2022/09/21/2022.09.20.508789.full.pdf
- OA Status
- green
- Cited By
- 2
- References
- 97
- Related Works
- 10
- OpenAlex ID
- https://openalex.org/W4296707148
All OpenAlex metadata
Raw OpenAlex JSON
- OpenAlex ID
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https://openalex.org/W4296707148Canonical identifier for this work in OpenAlex
- DOI
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https://doi.org/10.1101/2022.09.20.508789Digital Object Identifier
- Title
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Mitochondrial protein import clogging as a mechanism of diseaseWork title
- Type
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preprintOpenAlex work type
- Language
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enPrimary language
- Publication year
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2022Year of publication
- Publication date
-
2022-09-21Full publication date if available
- Authors
-
Liam P. Coyne, Xiaowen Wang, Jiyao Song, Ebbing P. de Jong, Karin Schneider, Paul T. Massa, Frank A. Middleton, Thomas Becker, Xin Jie ChenList of authors in order
- Landing page
-
https://doi.org/10.1101/2022.09.20.508789Publisher landing page
- PDF URL
-
https://www.biorxiv.org/content/biorxiv/early/2022/09/21/2022.09.20.508789.full.pdfDirect link to full text PDF
- Open access
-
YesWhether a free full text is available
- OA status
-
greenOpen access status per OpenAlex
- OA URL
-
https://www.biorxiv.org/content/biorxiv/early/2022/09/21/2022.09.20.508789.full.pdfDirect OA link when available
- Concepts
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Biology, Proteostasis, Mitochondrion, Cell biology, Intermembrane space, Translocase, Transport protein, ATP–ADP translocase, Mitochondrial biogenesis, Inner mitochondrial membrane, Biochemistry, Bacterial outer membrane, Chromosomal translocation, Gene, Escherichia coliTop concepts (fields/topics) attached by OpenAlex
- Cited by
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2Total citation count in OpenAlex
- Citations by year (recent)
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2024: 2Per-year citation counts (last 5 years)
- References (count)
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97Number of works referenced by this work
- Related works (count)
-
10Other works algorithmically related by OpenAlex
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