Control of mitochondrial morphology by a human mitofusin

Santel, Ansgar; Fuller, Margaret T.

doi:10.1242/jcs.114.5.867

Cited by 1,012Mitochondrial Function and PathologyAutophagy in Disease and TherapyCell death mechanisms and regulation

Control of mitochondrial morphology by a human mitofusin

Santel, Ansgar, Fuller, Margaret T.·2001·Journal of Cell Science

DOI 10.1242/jcs.114.5.867

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Abstract

Although changes in mitochondrial size and arrangement accompany both cellular differentiation and human disease, the mechanisms that mediate mitochondrial fusion, fission and morphogenesis in mammalian cells are not understood. We have identified two human genes encoding potential mediators of mitochondrial fusion. The mitofusins (Mfn1 and Mfn2) are homologs of the Drosophila protein fuzzy onion (Fzo) that associate with mitochondria and alter mitochondrial morphology when expressed by transient transfection in tissue culture cells. An internal region including a predicted bipartite transmembrane domain (TM) is sufficient to target Mfn2 to mitochondria and requires hydrophobic residues within the TM. Co-expression of Mfn2 with a dominant interfering mutant dynamin-related protein (Drp1K38A) proposed to block mitochondrial fission resulted in long mitochondrial filaments and networks. Formation of mitochondrial filaments and networks required a wild-type Mfn2 GTPase domain, suggesting that the Mfn2 GTPase regulates or mediates mitochondrial fusion and that mitofusins and dynamin related GTPases play opposing roles in mitochondrial fusion and fission in mammals, as in yeast.

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Mfn2 participates in the maintenance of mitochondrial tubules or networks in mammalian cells.
“Mitofusin-2 (Mfn2) participates in the maintenance of mitochondrial tubules or networks in mammalian cells (23)(24)(25).”
From Expression of <i>Mfn2</i>, the Charcot-Marie-Tooth Neuropathy Type 2A Gene, in Human Skeletal Muscle (2005) · cites this paper as [24]
Mfn2 is a mitochondrial membrane protein that participates in mitochondrial fusion in mammalian cells.
“We show that Mfn2 (mitofusin 2), a mitochondrial membrane protein that participates in mitochondrial fusion in mammalian cells, is induced during myogenesis and contributes to the maintenance and operation of the mitochondrial network.”
From Mitofusin-2 Determines Mitochondrial Network Architecture and Mitochondrial Metabolism (2003) · cites this paper as [22]

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Authors: Santel, A., & Fuller, M. T.
Affiliations: Stanford Medicine (US)
ORCID: Fuller, Margaret T.
Venue: Journal of Cell Science
Publisher: The Company of Biologists
Published: 2001-03-01
Volume: 114
Issue: 5
Pages: 867-874
ISSN: 0021-9533 (ISSN-L)
1477-9137
DOI: 10.1242/jcs.114.5.867
OpenAlex: W2144846851
Cited by: 1,012
References: 33
Citations by year: 2026: 14 · 2025: 38 · 2024: 45 · 2023: 50 · 2022: 50 · 2021: 45 · 2020: 68 · 2019: 73 · 2018: 66 · 2017: 52 · 2016: 42 · 2015: 41 · 2014: 39 · 2013: 43 · 2012: 57
FWCI: 8.96
Type: journal article
Language: en
Open access: closed
Licence: www.biologists.com/user-licence-1-1/ (vor)
Locations: Journal of Cell Science (publishedVersion)
PubMed (publishedVersion)
HighWire Press Open Archive (submittedVersion)
Funders: National Institutes of Health (5r01hd029194-05)
Eunice Kennedy Shriver National Institute of Child Health and Human Development (HD29194)
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 › Cell death mechanisms and regulation
Keywords: mitofusin 2, mitofusin 1, mitochondrial fusion, mitochondrial fission, mitochondrial morphology, mitochondrial network, mitochondrial targeting, Drp1, dynamin-related GTPase, GTPase domain, mitochondrial morphogenesis
MeSH terms: Animals, Cell Line, Fluorescent Antibody Technique, Humans, Mitochondria, Receptors, CXCR4, GTP Phosphohydrolases

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Control of mitochondrial morphology by a human mitofusin

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