Citations for
The abundance of the ARL2 GTPase and its GAP, ELMOD2, at mitochondria are modulated by the fusogenic activity of mitofusins and stressors.
Newman LE, Schiavon CR, Zhou C, Kahn RA.
PLoS One 12(4):e0175164. doi: 10.1371/journal.pone.0175164. eCollection 2017. 2017
Activation of Mitofusin2 by Smad2-RIN1 Complex during Mitochondrial Fusion.
Kumar S, Pan CC, Shah N, Wheeler SE, Hoyt KR, Hempel N, Mythreye K, Lee NY.
Mol Cell 62(4):520-31. doi: 10.1016/j.molcel.2016.04.010. Epub 2016 May 12. 2016
3MFN2, OPA1, SLC25A46
SLC25A46 is required for mitochondrial lipid homeostasis and cristae maintenance and is responsible for Leigh syndrome
Janer A, Prudent J, Paupe V, Fahiminiya S, Majewski J, Sgarioto N, Des Rosiers C, Forest A, Lin ZY, Gingras AC, Mitchell G, McBride HM, Shoubridge EA.
EMBO Mol Med. Sep 1;8(9):1019-38. doi: 10.15252/emmm.201506159. 2016
Distinct mechanisms controlling rough and smooth endoplasmic reticulum contacts with mitochondria.
Wang PT, Garcin PO, Fu M, Masoudi M, St-Pierre P, Panté N, Nabi IR.
J Cell Sci 128(15):2759-65. doi: 10.1242/jcs.171132. Epub 2015 Jun 11. 2015
HDAC6 maintains mitochondrial connectivity under hypoxic stress by suppressing MARCH5/MITOL dependent MFN2 degradation.
Kim HJ, Nagano Y, Choi SJ, Park SY, Kim H, Yao TP, Lee JY.
Biochem Biophys Res Commun 464(4):1235-1240. doi: 10.1016/j.bbrc.2015.07.111. Epub 2015 Jul 23. 2015
Role of Mitofusin-2 in High Mobility Group Box-1 Protein-Mediated Apoptosis of T Cells in Vitro.
Wu ZS, Yao YM, Hong GL, Xu XP, Liu Y, Dong N, Zheng JY, Lu ZQ, Zhao GJ, Zhu XM, Zhang QH, Sheng ZY.
Cell Physiol Biochem 33(3):769-783. [Epub ahead of print] 2014
Novel mitofusin 2 splice-site mutation causes Charcot-Marie-Tooth disease type 2 with prominent sensory dysfunction.
Martikainen MH, Kytövuori L, Majamaa K.
Neuromuscul Disord 24(4):360-4. doi: 10.1016/j.nmd.2014.01.007. Epub 2014 Jan 27. 2014
Role of mitofusin 2 (Mfn2) in controlling cellular proliferation.
Chen KH, Dasgupta A, Ding J, Indig FE, Ghosh P, Longo DL.
FASEB J 28(1):382-94. doi: 10.1096/fj.13-230037. Epub 2013 Sep 30. 2014
MITOL regulates endoplasmic reticulum-mitochondria contacts via Mitofusin2.
Sugiura A, Nagashima S, Tokuyama T, Amo T, Matsuki Y, Ishido S, Kudo Y, McBride HM, Fukuda T, Matsushita N, Inatome R, Yanagi S.
Mol Cell 51(1):20-34. doi: 10.1016/j.molcel.2013.04.023. Epub 2013 May 30. 2013
10MFN1, MFN2
Mitochondrial fusion directs cardiomyocyte differentiation via calcineurin and Notch signaling.
Kasahara A, Cipolat S, Chen Y, Dorn GW 2nd, Scorrano L.
Science 342(6159):734-7. doi: 10.1126/science.1241359. Epub 2013 Oct 3. 2013
Mitochondrial protein mitofusin 2 is required for NLRP3 inflammasome activation after RNA virus infection.
Ichinohe T, Yamazaki T, Koshiba T, Yanagi Y.
Proc Natl Acad Sci U S A 110(44):17963-8. doi: 10.1073/pnas.1312571110. Epub 2013 Oct 14. 2013
A form of mitofusin 2 (Mfn2) lacking the transmembrane domains and the COOH-terminal end stimulates metabolism in muscle and liver cells.
Segalés J, Paz JC, Hernández-Alvarez MI, Sala D, Muñoz JP, Noguera E, Pich S, Palacín M, Enríquez JA, Zorzano A.
Am J Physiol Endocrinol Metab 305(10):E1208-21. doi: 10.1152/ajpendo.00546.2012. Epub 2013 Aug 13. 2013
Mitofusin 2 in POMC neurons connects ER stress with leptin resistance and energy imbalance.
Schneeberger M, Dietrich MO, Sebastián D, Imbernón M, Castaño C, Garcia A, Esteban Y, Gonzalez-Franquesa A, Rodríguez IC, Bortolozzi A, Garcia-Roves PM, Gomis R, Nogueiras R, Horvath TL, Zorzano A, Claret M.
Cell 155(1):172-87. doi: 10.1016/j.cell.2013.09.003. 2013
Low expression of Mfn2 is associated with mitochondrial damage and apoptosis in the placental villi of early unexplained miscarriage.
Pang W, Zhang Y, Zhao N, Darwiche SS, Fu X, Xiang W.
Placenta 34(7):613-8. doi: 10.1016/j.placenta.2013.03.013. Epub 2013 Apr 17. 2013
15MFN2, SLC2A4
Overexpression of mitofusin 2 improves translocation of glucose transporter 4 in skeletal muscle of high‑fat diet‑fed rats through AMP‑activated protein kinase signaling.
Kong D, Song G, Wang C, Ma H, Ren L, Nie Q, Zhang X, Gan K.
Mol Med Rep 8(1):205-10. doi: 10.3892/mmr.2013.1457. Epub 2013 May 2. 2013
16MFN2, SP1
The promoter activity of human Mfn2 depends on Sp1 in vascular smooth muscle cells.
Sorianello E, Soriano FX, Fernández-Pascual S, Sancho A, Naon D, Vila-Caballer M, González-Navarro H, Portugal J, Andrés V, Palacín M, Zorzano A.
Cardiovasc Res 94(1):38-47. Epub 2012 Jan 17. 2012
17MFN1, MFN2
Mitofusin 1 inhibits an apoptosis-associated amino-terminal conformational change in Bax, but not its mitochondrial translocation, in a GTPase-dependent manner.
Ryu SW, Choi K, Park JH, Park YM, Kim S, Choi C.
Cancer Lett 323(1):62-8. Epub 2012 Apr 4. 2012
Mitofusin-2 independent juxtaposition of endoplasmic reticulum and mitochondria: an ultrastructural study.
Cosson P, Marchetti A, Ravazzola M, Orci L.
PLoS One 7(9):e46293. doi: 10.1371/journal.pone.0046293. Epub 2012 Sep 28. 2012
Central role of mitofusin 2 in autophagosome-lysosome fusion in cardiomyocytes.
Zhao T, Huang X, Han L, Wang X, Cheng H, Zhao Y, Chen Q, Chen J, Cheng H, Xiao R, Zheng M.
J Biol Chem 287(28):23615-25. doi: 10.1074/jbc.M112.379164. Epub 2012 May 22. 2012
Mitofusin2 mutations disrupt axonal mitochondrial positioning and promote axon degeneration.
Misko AL, Sasaki Y, Tuck E, Milbrandt J, Baloh RH.
J Neurosci 32(12):4145-55. doi: 10.1523/JNEUROSCI.6338-11.2012. 2012
Mitofusin 2 (Mfn2) links mitochondrial and endoplasmic reticulum function with insulin signaling and is essential for normal glucose homeostasis.
Sebastián D, Hernández-Alvarez MI, Segalés J, Sorianello E, Muñoz JP, Sala D, Waget A, Liesa M, Paz JC, Gopalacharyulu P, Orešič M, Pich S, Burcelin R, Palacín M, Zorzano A.
Proc Natl Acad Sci U S A 109(14):5523-8. doi: 10.1073/pnas.1108220109. Epub 2012 Mar 16. 2012
Parkin and mitofusins reciprocally regulate mitophagy and mitochondrial spheroid formation.
Ding WX, Guo F, Ni HM, Bockus A, Manley S, Stolz DB, Eskelinen EL, Jaeschke H, Yin XM.
J Biol Chem 287(50):42379-88. doi: 10.1074/jbc.M112.413682. Epub 2012 Oct 24. 2012
Loss of mitofusin 2 promotes endoplasmic reticulum stress.
Ngoh GA, Papanicolaou KN, Walsh K.
J Biol Chem 287(24):20321-32. doi: 10.1074/jbc.M112.359174. Epub 2012 Apr 17. 2012
Loss of Mfn2 results in progressive, retrograde degeneration of dopaminergic neurons in the nigrostriatal circuit.
Pham AH, Meng S, Chu QN, Chan DC.
Hum Mol Genet 21(22):4817-26. doi: 10.1093/hmg/dds311. Epub 2012 Jul 31. 2012
Mitofusin 2 is necessary for striatal axonal projections of midbrain dopamine neurons.
Lee S, Sterky FH, Mourier A, Terzioglu M, Cullheim S, Olson L, Larsson NG.
Hum Mol Genet 21(22):4827-35. doi: 10.1093/hmg/dds352. Epub 2012 Aug 21. 2012
26CMT2A2, MFN2
Developmental defects and neuromuscular alterations due to mitofusin 2 gene (MFN2) silencing in zebrafish: a new model for Charcot-Marie-Tooth type 2A neuropathy.
Vettori A, Bergamin G, Moro E, Vazza G, Polo G, Tiso N, Argenton F, Mostacciuolo ML.
Neuromuscul Disord 21(1):58-67. Epub 2010 Oct 14. 2011
Mitofusin-2 maintains mitochondrial structure and contributes to stress-induced permeability transition in cardiac myocytes.
Papanicolaou KN, Khairallah RJ, Ngoh GA, Chikando A, Luptak I, O'Shea KM, Riley DD, Lugus JJ, Colucci WS, Lederer WJ, Stanley WC, Walsh K.
Mol Cell Biol 31(6):1309-28. Epub 2011 Jan 18. 2011
Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage.
Shirendeb U, Reddy AP, Manczak M, Calkins MJ, Mao P, Tagle DA, Reddy PH.
Hum Mol Genet 20(7):1438-55. Epub 2011 Jan 21. 2011
Mitofusin 2 regulates STIM1 migration from the Ca2+ store to the plasma membrane in cells with depolarized mitochondria.
Singaravelu K, Nelson C, Bakowski D, de Brito OM, Ng SW, Di Capite J, Powell T, Scorrano L, Parekh AB.
J Biol Chem 286(14):12189-201. Epub 2011 Jan 10. 2011
30CMT2A2, MFN2
MFN2 mutations cause severe phenotypes in most patients with CMT2A.
Feely SM, Laura M, Siskind CE, Sottile S, Davis M, Gibbons VS, Reilly MM, Shy ME.
Neurology 76(20):1690-6. Epub 2011 Apr 20. 2011
31CMT2A2, MFN2
Characterizing the phenotypic manifestations of MFN2 R104W mutation in Charcot-Marie-Tooth type 2.
Genari AB, Borghetti VH, Gouvêa SP, Bueno KC, dos Santos PL, dos Santos AC, Barreira AA, Lourenço CM, Marques W Jr.
Neuromuscul Disord 21(6):428-32. Epub 2011 Apr 29. 2011
32CMT2A2, MFN2
Recessive axonal Charcot-Marie-Tooth disease due to compound heterozygous mitofusin 2 mutations.
Polke JM, Laurá M, Pareyson D, Taroni F, Milani M, Bergamin G, Gibbons VS, Houlden H, Chamley SC, Blake J, Devile C, Sandford R, Sweeney MG, Davis MB, Reilly MM.
Neurology 77(2):168-73. Epub 2011 Jun 29. 2011
33MFN1, MFN2
Mitofusins are required for angiogenic function and modulate different signaling pathways in cultured endothelial cells.
Lugus JJ, Ngoh GA, Bachschmid MM, Walsh K.
J Mol Cell Cardiol 51(6):885-93. Epub 2011 Aug 2. 2011
34MFN1, MFN2
Mutations in PINK1 and Parkin impair ubiquitination of Mitofusins in human fibroblasts.
Rakovic A, Grünewald A, Kottwitz J, Brüggemann N, Pramstaller PP, Lohmann K, Klein C.
PLoS One 6(3):e16746. 2011
35CMT2A2, MFN2
Phenotypic spectrum of MFN2 mutations in the Spanish population.
Casasnovas C, Banchs I, Cassereau J, Gueguen N, Chevrollier A, Martínez-Matos JA, Bonneau D, Volpini V.
J Med Genet 47(4):249-56. Epub 2009 Nov 3. 2010
36CMT2A2, MFN2
MFN2 point mutations occur in 3.4% of Charcot-Marie-Tooth families. An investigation of 232 Norwegian CMT families.
Braathen GJ, Sand JC, Lobato A, Høyer H, Russell MB.
BMC Med Genet 11(1):48.PMID: 20350294 2010
Mitofusin 2 is necessary for transport of axonal mitochondria and interacts with the Miro/Milton complex.
Misko A, Jiang S, Wegorzewska I, Milbrandt J, Baloh RH.
J Neurosci 30(12):4232-40.PMID: 20335458 2010
Trichoplein/mitostatin regulates endoplasmic reticulum-mitochondria juxtaposition.
Cerqua C, Anesti V, Pyakurel A, Liu D, Naon D, Wiche G, Baffa R, Dimmer KS, Scorrano L.
EMBO Rep 11(11):854-60. Epub 2010 Oct 8. 2010
Mitofusin-2 is a novel direct target of p53.
Wang W, Cheng X, Lu J, Wei J, Fu G, Zhu F, Jia C, Zhou L, Xie H, Zheng S.
Biochem Biophys Res Commun 400(4):587-92. Epub 2010 Sep 6. 2010
Mitofusin 1 and mitofusin 2 are ubiquitinated in a PINK1/parkin-dependent manner upon induction of mitophagy.
Gegg ME, Cooper JM, Chau KY, Rojo M, Schapira AH, Taanman JW.
Hum Mol Genet 19(24):4861-70. Epub 2010 Sep 24. 2010
41MFN1, MFN2
Mitochondrial fission controls DNA fragmentation by regulating endonuclease G.
Li J, Zhou J, Li Y, Qin D, Li P.
Free Radic Biol Med 49(4):622-31. Epub 2010 Jun 1. 2010
Mitofusin 2 inhibits mitochondrial antiviral signaling.
Yasukawa K, Oshiumi H, Takeda M, Ishihara N, Yanagi Y, Seya T, Kawabata S, Koshiba T.
Sci Signal 2(84):ra47.PMID: 19690333 2009
Mitofusin 2 builds a bridge between ER and mitochondria.
Merkwirth C, Langer T.
Cell 135(7):1165-7. 2008
Mitofusin 2 tethers endoplasmic reticulum to mitochondria.
de Brito OM, Scorrano L.
Nature 456(7222):605-10. 2008
Mutated mitofusin 2 presents with intrafamilial variability and brain mitochondrial dysfunction.
Del Bo R, Moggio M, Rango M, Bonato S, D'Angelo MG, Ghezzi S, Airoldi G, Bassi MT, Guglieri M, Napoli L, Lamperti C, Corti S, Federico A, Bresolin N, Comi GP.
Neurology 71(24):1959-66. Epub 2008 Oct 22. 2008
Identification of a novel mitochondrial complex containing mitofusin 2 and stomatin-like protein 2.
Hajek P, Chomyn A, Attardi G.
J Biol Chem 282(8):5670-81. Epub 2006 Nov 22. 2007
47MFN2, CMT2A2
Mitochondrial GTPase mitofusin 2 mutations in Korean patients with Charcot-Marie-Tooth neuropathy type 2.
Cho HJ, Sung D, Kim B, Ki CS.
Clin Genet 71(3):267-72. 2007
Mitochondrial fusion protects against neurodegeneration in the cerebellum.
Chen H, McCaffery JM, Chan DC.
Cell 130(3):548-62. 2007
Mitofusin 2 protects cerebellar granule neurons against injury-induced cell death.
Jahani-Asl A, Cheung EC, Neuspiel M, MacLaurin JG, Fortin A, Park DS, McBride HM, Slack RS.
J Biol Chem 282(33):23788-98. Epub 2007 May 30. 2007
50CMT2A2, MFN2
Mitochondrial coupling defect in Charcot-Marie-Tooth type 2A disease.
Loiseau D, Chevrollier A, Verny C, Guillet V, Gueguen N, Pou de Crescenzo MA, Ferré M, Malinge MC, Guichet A, Nicolas G, Amati-Bonneau P, Malthièry Y, Bonneau D, Reynier P.
Ann Neurol 61(4):315-23. 2007
Mitochondrial clustering induced by overexpression of the mitochondrial fusion protein Mfn2 causes mitochondrial dysfunction and cell death.
Huang P, Yu T, Yoon Y.
Eur J Cell Biol 86(6):289-302. Epub 2007 May 25. 2007
MARCH-V is a novel mitofusin 2- and Drp1-binding protein able to change mitochondrial morphology.
Nakamura N, Kimura Y, Tokuda M, Honda S, Hirose S.
EMBO Rep 7(10):1019-22. Epub 2006 Aug 25. 2006
Axonal neuropathy with optic atrophy is caused by mutations in mitofusin 2.
Zuchner S, De Jonghe P, Jordanova A, Claeys KG, Guergueltcheva V, Cherninkova S, Hamilton SR, Van Stavern G, Krajewski KM, Stajich J, Tournev I, Verhoeven K, Langerhorst CT, de Visser M, Baas F, Bird T, Timmerman V, Shy M, Vance JM.
Ann Neurol 59(2):276-81. 2006
54CMT2A2, MFN2
Mitochondrial GTPase mitofusin 2 mutation in Charcot-Marie-Tooth neuropathy type 2A.
Kijima K, Numakura C, Izumino H, Umetsu K, Nezu A, Shiiki T, Ogawa M, Ishizaki Y, Kitamura T, Shozawa Y, Hayasaka K.
Hum Genet 116(1-2):23-7. Epub 2004 Nov 11. 2005
55MFN2, CMT2A2
The Charcot-Marie-Tooth type 2A gene product, Mfn2, up-regulates fuel oxidation through expression of OXPHOS system.
Pich S, Bach D, Briones P, Liesa M, Camps M, Testar X, Palacin M, Zorzano A.
Hum Mol Genet 14(11):1405-15. Epub 2005 Apr 13. 2005
56MFN2, CMT2A2
Emerging functions of mammalian mitochondrial fusion and fission.
Chen H, Chan DC.
Hum Mol Genet 14 Spec No. 2:R283-9. 2005
Expression of Mfn2, the Charcot-Marie-Tooth neuropathy type 2A gene, in human skeletal muscle: effects of type 2 diabetes, obesity, weight loss, and the regulatory role of tumor necrosis factor alpha and interleukin-6.
Bach D, Naon D, Pich S, Soriano FX, Vega N, Rieusset J, Laville M, Guillet C, Boirie Y, Wallberg-Henriksson H, Manco M, Calvani M, Castagneto M, Palacin M, Mingrone G, Zierath JR, Vidal H, Zorzano A.
Diabetes 54(9):2685-93. 2005
58MFN1, MFN2
Mitofusins 1/2 and ERRalpha expression are increased in human skeletal muscle after physical exercise.
Cartoni R, Léger B, Hock MB, Praz M, Crettenand A, Pich S, Ziltener JL, Luthi F, Dériaz O, Zorzano A, Gobelet C, Kralli A, Russell AP.
J Physiol 567(Pt 1):349-58. Epub 2005 Jun 16. 2005
59MFN2, CMT2A2
Mutations in the mitochondrial GTPase mitofusin 2 cause Charcot-Marie-Tooth neuropathy type 2A.
Zuchner S, Mersiyanova IV, Muglia M, Bissar-Tadmouri N, Rochelle J, Dadali EL, Zappia M, Nelis E, Patitucci A, Senderek J, Parman Y, Evgrafov O, Jonghe PD, Takahashi Y, Tsuji S, Pericak-Vance MA, Quattrone A, Battologlu E, Polyakov AV, Timmerman V, Schroder JM, Vance JM.
Nat Genet 36(5):449-51. Epub 2004 Apr 04. No abstract available. 2004
60OPA1, MFN1, MFN2
OPA1 requires mitofusin 1 to promote mitochondrial fusion.
Cipolat S, Martins de Brito O, Dal Zilio B, Scorrano L.
Proc Natl Acad Sci U S A 101(45):15927-32. Epub 2004 Oct 27. 2004
Mitofusin-2 determines mitochondrial network architecture and mitochondrial metabolism. A novel regulatory mechanism altered in obesity.
Bach D, Pich S, Soriano FX, Vega N, Baumgartner B, Oriola J, Daugaard JR, Lloberas J, Camps M, Zierath JR, Rabasa-Lhoret R, Wallberg-Henriksson H, Laville M, Palacin M, Vidal H, Rivera F, Brand M, Zorzano A.
J Biol Chem 278(19):17190-7. Epub 2003 Feb 21. 2003
62MFN1, MFN2
Membrane topology and mitochondrial targeting of mitofusins, ubiquitous mammalian homologs of the transmembrane GTPase Fzo.
Rojo M, Legros F, Chateau D, Lombes A.
J Cell Sci 115(Pt 8):1663-74. 2002
Spatial and temporal association of Bax with mitochondrial fission sites, Drp1, and Mfn2 during apoptosis.
Karbowski M, Lee YJ, Gaume B, Jeong SY, Frank S, Nechushtan A, Santel A, Fuller M, Smith CL, Youle RJ.
J Cell Biol 159(6):931-8. 2002
64MFN1, MFN2
Control of mitochondrial morphology by a human mitofusin.
Santel A, Fuller MT.
J Cell Sci 114(Pt 5):867-74. 2001
Prediction of the coding sequences of unidentified human genes. VI. the coding sequences of 80 new genes (KIAA0201-KIAA0280) deduced by analysis of cDNA clones from cell line KG-1 and brain.
Nagase T, et al.
DNA Res 3 : 321-329,341-54. 1996