MFN1

MFN1
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	1T3J
Identifiers
Aliases	MFN1 , hfzo1, hfzo2, mitofusin 1
External IDs	OMIM: 608506 MGI: 1914664 HomoloGene: 11481 GeneCards: MFN1
Gene location (Human)
Chr.	Chromosome 3 (human)
End	179,394,936 bp
Gene location (Mouse)
Chr.	Chromosome 3 (mouse)
End	32,633,388 bp
RNA expression pattern
	Top expressed in
	secondary oocyte; ; Achilles tendon; ; body of pancreas; ; gastric mucosa; ; right uterine tube; ; canal of the cervix; ; skin of abdomen; ; minor salivary gland; ; body of stomach; ; left ventricle;
	Top expressed in
	myocardium of ventricle; ; digastric muscle; ; right ventricle; ; extraocular muscle; ; soleus muscle; ; extensor digitorum longus muscle; ; thoracic diaphragm; ; sternocleidomastoid muscle; ; tibialis anterior muscle; ; plantaris muscle;
	More reference expression data
	n/a
Gene ontology
Molecular function	nucleotide binding ; GTP binding ; protein binding ; hydrolase activity ; GTPase activity ;
Cellular component	cytoplasm ; mitochondrial outer membrane ; mitochondrion ; membrane ; integral component of mitochondrial outer membrane ; outer mitochondrial membrane protein complex ; integral component of membrane ; intrinsic component of mitochondrial outer membrane ;
Biological process	mitochondrial fusion ; macroautophagy ; GTP metabolic process ; mitochondrion localization ;
	Sources:Amigo / QuickGO
Orthologs
	55669
	67414
	ENSG00000171109
	ENSMUSG00000027668
	Q8IWA4
	Q811U4
	NM_017927 ; NM_033540
	NM_024200
	NP_284941
	NP_077162
	Wikidata
View/Edit Human	View/Edit Mouse

Last updated December 30, 2023

Mitofusin-1 is a protein that in humans is encoded by the MFN1 gene.^[5]^[6]^[7]

The protein encoded by this gene is a mediator of mitochondrial fusion. This protein and mitofusin 2 are homologs of the Drosophila protein fuzzy onion (Fzo). They are mitochondrial membrane proteins that interact with each other to facilitate mitochondrial targeting.^[7]

Related Research Articles

Mitofusin-2 is a protein that in humans is encoded by the MFN2 gene. Mitofusins are GTPases embedded in the outer membrane of the mitochondria. In mammals MFN1 and MFN2 are essential for mitochondrial fusion. In addition to the mitofusins, OPA1 regulates inner mitochondrial membrane fusion, and DRP1 is responsible for mitochondrial fission.

Periplakin is a protein that in humans is encoded by the PPL gene.

Chimerin 2 (beta-chimaerin) is a protein that in humans is encoded by the CHN2 gene.

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Tetraspanin-13 is a protein that in humans is encoded by the TSPAN13 gene.

ADP-ribosylation factor-related protein 1 is a protein that in humans is encoded by the ARFRP1 gene.

Zinc finger FYVE domain-containing protein 1 is a protein that in humans is encoded by the ZFYVE1 gene.

Protein Hook homolog 3 is a protein that in humans is encoded by the HOOK3 gene.

Myosin-Ib is a protein that in humans is encoded by the MYO1B gene.

Tetraspanin-6 is a protein that in humans is encoded by the TSPAN6 gene.

Tetraspanin-3 is a protein that in humans is encoded by the TSPAN3 gene.

Centrin-1 is a protein that in humans is encoded by the CETN1 gene. It belongs to the centrin family of proteins.

A disintegrin and metalloproteinase with thrombospondin motifs 12 is an enzyme that in humans is encoded by the ADAMTS12 gene.

Sorting nexin-4 is a protein that in humans is encoded by the SNX4 gene.

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Mitochondria are dynamic organelles with the ability to fuse and divide (fission), forming constantly changing tubular networks in most eukaryotic cells. These mitochondrial dynamics, first observed over a hundred years ago are important for the health of the cell, and defects in dynamics lead to genetic disorders. Through fusion, mitochondria can overcome the dangerous consequences of genetic malfunction. The process of mitochondrial fusion involves a variety of proteins that assist the cell throughout the series of events that form this process.

References

1 2 3 GRCh38: Ensembl release 89: ENSG00000171109 - Ensembl, May 2017
1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000027668 - Ensembl, May 2017
↑ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
↑ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
↑ Adams MD, Kerlavage AR, Fields C, Venter JC (Sep 1993). "3,400 new expressed sequence tags identify diversity of transcripts in human brain". Nat Genet. 4 (3): 256–67. doi:10.1038/ng0793-256. PMID 8358434. S2CID 20528590.
↑ Santel A, Fuller MT (Feb 2001). "Control of mitochondrial morphology by a human mitofusin". J Cell Sci. 114 (Pt 5): 867–74. doi: 10.1242/jcs.114.5.867 . PMID 11181170.
1 2 "Entrez Gene: MFN1 mitofusin 1".

Pawlikowska P, Orzechowski A (2007). "[Role of transmembrane GTPases in mitochondrial morphology and activity]". Postepy Biochem. 53 (1): 53–9. PMID 17718388.
Andersson B, Wentland MA, Ricafrente JY, et al. (1996). "A "double adaptor" method for improved shotgun library construction". Anal. Biochem. 236 (1): 107–13. doi:10.1006/abio.1996.0138. PMID 8619474.
Yu W, Andersson B, Worley KC, et al. (1997). "Large-Scale Concatenation cDNA Sequencing". Genome Res. 7 (4): 353–8. doi:10.1101/gr.7.4.353. PMC 139146 . PMID 9110174.
Hales KG, Fuller MT (1997). "Developmentally regulated mitochondrial fusion mediated by a conserved, novel, predicted GTPase". Cell. 90 (1): 121–9. doi: 10.1016/S0092-8674(00)80319-0 . PMID 9230308. S2CID 15162087.
Chung JG, Yeh KT, Wu SL, et al. (2002). "Novel transmembrane GTPase of non-small cell lung cancer identified by mRNA differential display". Cancer Res. 61 (24): 8873–9. PMID 11751411.
Rojo M, Legros F, Chateau D, Lombès A (2003). "Membrane topology and mitochondrial targeting of mitofusins, ubiquitous mammalian homologs of the transmembrane GTPase Fzo". J. Cell Sci. 115 (Pt 8): 1663–74. doi:10.1242/jcs.115.8.1663. PMID 11950885.
Legros F, Lombès A, Frachon P, Rojo M (2003). "Mitochondrial Fusion in Human Cells Is Efficient, Requires the Inner Membrane Potential, and Is Mediated by Mitofusins". Mol. Biol. Cell. 13 (12): 4343–54. doi:10.1091/mbc.E02-06-0330. PMC 138638 . PMID 12475957.
Strausberg RL, Feingold EA, Grouse LH, et al. (2003). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–903. Bibcode:2002PNAS...9916899M. doi: 10.1073/pnas.242603899 . PMC 139241 . PMID 12477932.
Santel A, Frank S, Gaume B, et al. (2004). "Mitofusin-1 protein is a generally expressed mediator of mitochondrial fusion in mammalian cells". J. Cell Sci. 116 (Pt 13): 2763–74. doi:10.1242/jcs.00479. PMID 12759376. S2CID 6661619.
Ota T, Suzuki Y, Nishikawa T, et al. (2004). "Complete sequencing and characterization of 21,243 full-length human cDNAs". Nat. Genet. 36 (1): 40–5. doi: 10.1038/ng1285 . PMID 14702039.
Gerhard DS, Wagner L, Feingold EA, et al. (2004). "The Status, Quality, and Expansion of the NIH Full-Length cDNA Project: The Mammalian Gene Collection (MGC)". Genome Res. 14 (10B): 2121–7. doi:10.1101/gr.2596504. PMC 528928 . PMID 15489334.
Cipolat S, Martins de Brito O, Dal Zilio B, Scorrano L (2004). "OPA1 requires mitofusin 1 to promote mitochondrial fusion". Proc. Natl. Acad. Sci. U.S.A. 101 (45): 15927–32. Bibcode:2004PNAS..10115927C. doi: 10.1073/pnas.0407043101 . PMC 528769 . PMID 15509649.
Cartoni R, Léger B, Hock MB, et al. (2005). "Mitofusins 1/2 and ERRα expression are increased in human skeletal muscle after physical exercise". J. Physiol. 567 (Pt 1): 349–58. doi:10.1113/jphysiol.2005.092031. PMC 1474174 . PMID 15961417.

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[refGRCh38Ensembl-1] 1 2 3 GRCh38: Ensembl release 89: ENSG00000171109 - Ensembl, May 2017

[refGRCm38Ensembl-2] 1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000027668 - Ensembl, May 2017

[3] "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[4] "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[pmid8358434-5] Adams MD, Kerlavage AR, Fields C, Venter JC (Sep 1993). "3,400 new expressed sequence tags identify diversity of transcripts in human brain". Nat Genet. 4 (3): 256–67. doi:10.1038/ng0793-256. PMID 8358434. S2CID 20528590.

[pmid11181170-6] Santel A, Fuller MT (Feb 2001). "Control of mitochondrial morphology by a human mitofusin". J Cell Sci. 114 (Pt 5): 867–74. doi: 10.1242/jcs.114.5.867 . PMID 11181170.

[entrez-7] 1 2 "Entrez Gene: MFN1 mitofusin 1".

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[5]

[6]

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MFN1

Related Research Articles

References

Further reading