Cited by 1,300Mitochondrial Function and PathologyAutophagy in Disease and TherapyATP Synthase and ATPases Research

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OPA1 requires mitofusin 1 to promote mitochondrial fusion

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Cipolat, Sara, de Brito, Olga Martins, Dal Zilio, Barbara, Scorrano, Luca·2004·Proceedings of the National Academy of Sciences

DOI 10.1073/pnas.0407043101PMC PMC528769

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Abstract

The regulated equilibrium between mitochondrial fusion and fission is essential to maintain integrity of the organelle. Mechanisms of mitochondrial fusion are largely uncharacterized in mammalian cells. It is unclear whether OPA1, a dynamin-related protein of the inner membrane mutated in autosomal dominant optic atrophy, participates in fusion or fission. OPA1 promoted the formation of a branched network of elongated mitochondria, requiring the integrity of both its GTPase and C-terminal coiled-coil domain. Stable reduction of OPA1 levels by RNA interference resulted in small, fragmented, and scattered mitochondria. Levels of OPA1 did not affect mitochondrial docking, but they correlated with the extent of fusion as measured by polyethylene glycol mitochondrial fusion assays. A genetic analysis proved that OPA1 was unable to tubulate and fuse mitochondria lacking the outer membrane mitofusin 1 but not mitofusin 2. Our data show that OPA1 functionally requires mitofusin 1 to regulate mitochondrial fusion and reveal a specific functional difference between mitofusin 1 and 2.

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Authors: Cipolat, S., de Brito, O. M., Dal Zilio, B., & Scorrano, L.
Affiliations: Veneto Institute of Molecular Medicine (IT)
Dulbecco–Telethon Institute, Venetian Institute of Molecular Medicine, Via Orus 2, I-35129 Padua, Italy
ORCID: Scorrano, Luca
Venue: Proceedings of the National Academy of Sciences
Publisher: National Academy of Sciences
Published: 2004-10-27
Volume: 101
Issue: 45
Pages: 15927-15932
ISSN: 0027-8424 (ISSN-L)
1091-6490
DOI: 10.1073/pnas.0407043101
PMC: PMC528769
OpenAlex: W1989540421
Cited by: 1,300
References: 45
Citations by year: 2026: 46 · 2025: 76 · 2024: 64 · 2023: 74 · 2022: 75 · 2021: 88 · 2020: 90 · 2019: 82 · 2018: 73 · 2017: 59 · 2016: 66 · 2015: 50 · 2014: 58 · 2013: 53 · 2012: 63
FWCI: 10.44
Type: journal article
Language: en
Open access: green
europepmc.org
Locations: Proceedings of the National Academy of Sciences (publishedVersion)
Europe PMC (PubMed Central) (publishedVersion)
PubMed Central (publishedVersion)
PubMed (publishedVersion)
Padua Research Archive (University of Padova) (submittedVersion)
Topics: Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Autophagy in Disease and Therapy
Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › ATP Synthase and ATPases Research
Keywords: OPA1, mitofusin 1, mitofusin 2, mitochondrial fusion, mitochondrial fission, dynamin-related proteins, GTPase, coiled-coil domain, mitochondrial outer membrane, mitochondrial tubulation, autosomal dominant optic atrophy, mitochondrial morphology
MeSH terms: Animals, Cells, Cultured, Membrane Fusion, Mitochondria, Mice, Knockout, Gene Targeting, GTP Phosphohydrolases, RNA Interference, Mice, In Vitro Techniques

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OPA1 requires mitofusin 1 to promote mitochondrial fusion

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