MFN2 monoclonal antibody (M03), clone 4H8
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More Files
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Specification
Product Description
Mouse monoclonal antibody raised against a partial recombinant MFN2.
Immunogen
MFN2 (NP_055689, 661 a.a. ~ 757 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
FKRQFVEHASEKLQLVISYTGSNCSHQVQQELSGTFAHLCQQVDVTRENLEQEIAAMNKKIEVLDSLQSKAKLLRNKAGWLDSELNMFTHQYLQPSR
Host
Mouse
Reactivity
Human, Rat
Interspecies Antigen Sequence
Mouse (93); Rat (93)
Isotype
IgG2a Kappa
Quality Control Testing
Antibody Reactive Against Recombinant Protein.
Western Blot detection against Immunogen (36.41 KDa) .
Storage Buffer
In 1x PBS, pH 7.4
Storage Instruction
Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
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Applications
Western Blot (Cell lysate)
MFN2 monoclonal antibody (M03), clone 4H8 Western Blot analysis of MFN2 expression in HeLa ( Cat # L013V1 ).Western Blot (Cell lysate)
MFN2 monoclonal antibody (M03), clone 4H8. Western Blot analysis of MFN2 expression in PC-12 ( Cat # L012V1 ).Western Blot (Transfected lysate)
Western Blot analysis of MFN2 expression in transfected 293T cell line by MFN2 monoclonal antibody (M03), clone 4H8.
Lane 1: MFN2 transfected lysate(86.4 KDa).
Lane 2: Non-transfected lysate.
Western Blot (Recombinant protein)
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Immunoperoxidase of monoclonal antibody to MFN2 on formalin-fixed paraffin-embedded human heart. [antibody concentration 5 ug/ml]Sandwich ELISA (Recombinant protein)
Detection limit for recombinant GST tagged MFN2 is approximately 1ng/ml as a capture antibody.ELISA
RNAi Knockdown (Antibody validated)
Western blot analysis of MFN2 over-expressed 293 cell line, cotransfected with MFN2 Validated Chimera RNAi ( Cat # H00009927-R01V ) (Lane 2) or non-transfected control (Lane 1). Blot probed with MFN2 monoclonal antibody (M03), clone 4H8 (Cat # H00009927-M03 ). GAPDH ( 36.1 kDa ) used as specificity and loading control. -
Gene Info — MFN2
Entrez GeneID
9927GeneBank Accession#
NM_014874Protein Accession#
NP_055689Gene Name
MFN2
Gene Alias
CMT2A, CMT2A2, CPRP1, HSG, KIAA0214, MARF
Gene Description
mitofusin 2
Gene Ontology
HyperlinkGene Summary
This gene encodes a mitochondrial membrane protein that participates in mitochondrial fusion and contributes to the maintenance and operation of the mitochondrial network. This protein is involved in the regulation of vascular smooth muscle cell proliferation, and it may play a role in the pathophysiology of obesity. Mutations in this gene cause Charcot-Marie-Tooth disease type 2A2, and hereditary motor and sensory neuropathy VI, which are both disorders of the peripheral nervous system. Defects in this gene have also been associated with early-onset stroke. Two transcript variants encoding the same protein have been identified. [provided by RefSeq
Other Designations
OTTHUMP00000002509|hyperplasia suppressor|mitochondrial assembly regulatory factor|mitofusin-2|transmembrane GTPase MFN2
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Interactome
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Disease
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Publication Reference
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Splice variants of mitofusin 2 shape the endoplasmic reticulum and tether it to mitochondria.
Déborah Naón, María Isabel Hernández-Alvarez, Satoko Shinjo, Milosz Wieczor, Saska Ivanova, Olga Martins de Brito, Albert Quintana, Juan Hidalgo, Manuel Palacín, Pilar Aparicio, Juan Castellanos, Luis Lores, David Sebastián, Sonia Fernández-Veledo, Joan Vendrell, Jorge Joven, Modesto Orozco, Antonio Zorzano, Luca Scorrano.
Science 2023 Jun; 380(6651):eadh9351.
Application:WB-Ti, Human, Human liver, human muscle, human WAT.
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Dietary nitrate preserves mitochondrial bioenergetics and mitochondrial protein synthesis rates during short-term immobilization in mice.
Heather L Petrick, Rachel M Handy, Bayley Vachon, Sara M Frangos, Andrew M Holwerda, Annemarie P Gijsen, Joan M Senden, Luc J C van Loon, Graham P Holloway.
The Journal of Physiology 2023 Jun; [Epub]:0.
Application:WB-Ti, Mouse, Mouse gastrocnemius.
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Inhibition of the mitochondria-shaping protein Opa1 restores sensitivity to Gefitinib in a lung adenocarcinomaresistant cell line.
Masafumi Noguchi, Susumu Kohno, Anna Pellattiero, Yukino Machida, Keitaro Shibata, Norihito Shintani, Takashi Kohno, Noriko Gotoh, Chiaki Takahashi, Atsushi Hirao0, Luca Scorrano, Atsuko Kasahara.
Cell Death & Disease 2023 Apr; 14(4):241.
Application:WB-Ce, Human, PC9, PC9M2 cells.
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Cardiolipin-Mediated Alleviation of Mitochondrial Dysfunction Is a Neuroprotective Effect of Statin in Animal Model of Ischemic Stroke.
Deepaneeta Sarmah, Abhishek Sarkar, Aishika Datta, Bijoyani Ghosh, Nikita Rana, Shubhrakanta Sahu, Vishal Gupta, Vrushali Thongire, Antra Chaudhary, Namrata Vadak, Harpreet Kaur, Swapnil Raut, Upasna Singh, Anupom Borah, Pallab Bhattacharya.
ACS Chemical Neuroscience 2023 Jan; 14(4):709.
Application:WB-Ti, Rat, Rat Cortex.
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Chronic social stress disrupts the intracellular redistribution of brain hexokinase 3 induced by shifts in peripheral glucose levels.
Michael A van der Kooij, Liliana Rojas-Charry, Maryam Givehchi, Christina Wolf, Diones Bueno, Sabine Arndt, Stefan Tenzer, Lorenzo Mattioni, Giulia Treccani, Annika Hasch, Michael J Schmeisser, Caterina Vianello, Marta Giacomello, Axel Methner.
Journal of Molecular Medicine (Berlin, Germany) 2022 Oct; 100(10):1441.
Application:WB, Mouse, Brain tissue.
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GDAP1 loss of function inhibits the mitochondrial pyruvate dehydrogenase complex by altering the actin cytoskeleton.
Christina Wolf, Alireza Pouya, Sara Bitar, Annika Pfeiffer, Diones Bueno, Liliana Rojas-Charry, Sabine Arndt, David Gomez-Zepeda, Stefan Tenzer, Federica Dal Bello, Caterina Vianello, Sandra Ritz, Jonas Schwirz, Kristina Dobrindt, Michael Peitz, Eva-Maria Hanschmann, Pauline Mencke, Ibrahim Boussaad, Marion Silies, Oliver Brüstle, Marta Giacomello, Rejko Krüger, Axel Methner.
Communications Biology 2022 Jun; 5(1):541.
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Myoglobin expression by alternative transcript in different mesenchymal stem cells compartments.
Rosella Scrima, Francesca Agriesti1, Consiglia Pacelli, Claudia Piccoli, Pietro Pucci, Angela Amoresano, Olga Cela, Luigi Nappi, Tiziana Tataranni, Giorgio Mori, Pietro Formisano and Nazzareno Capitanio.
Stem Cell Research & Therapy 2022 May; 13(1):209.
Application:WB-Ce, Human, MSCs.
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Dynamic O-GlcNAcylation coordinates ferritinophagy and mitophagy to activate ferroptosis.
Fan Yu, Qianping Zhang, Hanyu Liu, Jinming Liu, Song Yang, Xiaofan Luo, Wei Liu, Hao Zheng, Qiqi Liu, Yunxi Cui, Guo Chen, Yanjun Li, Xinglu Huang, Xiyun Yan, Jun Zhou and Quan Chen.
Cell Discovery 2022 May; 8(1):40.
Application:WB, Human, U2OS cells.
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Cisplatin resistance can be curtailed by blunting Bnip3-mediated mitochondrial autophagy.
Caterina Vianello, Veronica Cocetta, Daniela Catanzaro, Gerald W Dorn 2nd, Angelo De Milito, Flavio Rizzolio, Vincenzo Canzonieri, Erika Cecchin, Rossana Roncato, Giuseppe Toffoli, Vincenzo Quagliariello, Annabella Di Mauro, Simona Losito, Nicola Maurea, Scaffa Cono, Gabriele Sales, Luca Scorrano, Marta Giacomello, Monica Montopoli.
Cell Death & Disease 2022 Apr; 13(4):398.
Application:WB-Ce, Human, 2008, C13, U2OS, U2OS-PT cells.
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Vmp1, Vps13D, and Marf/Mfn2 function in a conserved pathway to regulate mitochondria and ER contact in development and disease.
James L Shen, Tina M Fortier, Yan G Zhao, Ruoxi Wang, Margit Burmeister, Eric H Baehrecke.
Current Biology : CB 2021 Jul; 31(14):3028.
Application:WB-Ce, Human, Human fibroblasts.
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Exploiting pyocyanin to treat mitochondrial disease due to respiratory complex III dysfunction.
Roberta Peruzzo, Samantha Corrà, Roberto Costa, Michele Brischigliaro, Tatiana Varanita, Lucia Biasutto, Chiara Rampazzo, Daniele Ghezzi, Luigi Leanza, Mario Zoratti, Massimo Zeviani, Cristiano De Pittà, Carlo Viscomi, Rodolfo Costa, Ildikò Szabò.
Nature Communications 2021 Apr; 12(1):2103.
Application:WB-Tr, Mouse, MEFs.
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The Age-Sensitive Efficacy of Calorie Restriction on Mitochondrial Biogenesis and mtDNA Damage in Rat Liver.
Guglielmina Chimienti, Anna Picca, Flavio Fracasso, Francesco Russo, Antonella Orlando, Giuseppe Riezzo, Christiaan Leeuwenburgh, Vito Pesce, Angela Maria Serena Lezza.
International Journal of Molecular Sciences 2021 Feb; 22(4):1665.
Application:WB-Ti, Rat, Rat liver.
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Mitochondrial nucleoid morphology and respiratory function are altered in Drp1-deficient HeLa cells.
Ota A, Ishihara T, Ishihara N.
Journal of Biochemistry 2020 Mar; 167(3):287.
Application:WB-Tr, Human, HeLa cells.
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Alpha-Synuclein Preserves Mitochondrial Fusion and Function in Neuronal Cells.
Faustini G, Marchesan E, Zonta L, Bono F, Bottani E, Longhena F, Ziviani E, Valerio A, Bellucci A.
Oxidative Medicine and Cellular Longevity 2019 Nov; 2019:4246350.
Application:WB-Ce, Mouse, Mouse primary cortical neurons.
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A novel class of cardioprotective small-molecule PTP inhibitors.
Antonucci S, Di Sante M, Sileikyte J, Deveraux J, Bauer T, Bround MJ, Menabò R, Paillard M, Alanova P, Carraro M, Ovize M, Molkentin JD, Cohen M, Forte MA, Bernardi P, Di Lisa F, Murphy E.
Pharmacological Research 2020 Jan; 151:104548.
Application:WB-Ce, Rat, Neonatal rat ventricular myocytes.
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The Charcot-Marie Tooth Disease Mutation R94Q in MFN2 Decreases ATP Production but Increases Mitochondrial Respiration under Conditions of Mild Oxidative Stress.
Wolf C, Zimmermann R, Thaher O, Bueno D, Wüllner V, Schäfer MKE, Albrecht P, Methner A.
Cells 2019 Oct; 8(10):E1289.
Application:WB-Tr, Mouse, MEFs.
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Melatonin Effects on Non-Alcoholic Fatty Liver Disease Are Related to MicroRNA-34a-5p/Sirt1 Axis and Autophagy.
Stacchiotti A, Grossi I, García-Gómez R, Patel GA, Salvi A, Lavazza A, De Petro G, Monsalve M, Rezzani R.
Cells 2019 Sep; 8(9):E1053.
Application:IHC, Mouse, Liver.
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MSTO1 mutations cause mtDNA depletion, manifesting as muscular dystrophy with cerebellar involvement.
Donkervoort S, Sabouny R, Yun P, Gauquelin L, Chao KR, Hu Y, Al Khatib I, Töpf A, Mohassel P, Cummings BB, Kaur R, Saade D, Moore SA, Waddell LB, Farrar MA, Goodrich JK, Uapinyoying P, Chan SHS, Javed A, Leach ME, Karachunski P, Dalton J, Medne L, Harper A, Thompson C, Thiffault I, Specht S, Lamont RE, Saunders C, Racher H, Bernier FP, Mowat D, Witting N, Vissing J, Hanson R, Coffman KA, Hainlen M, Parboosingh JS, Carnevale A, Yoon G, Schnur RE; Care4Rare Canada Consortium, Boycott KM, Mah JK, S
Acta Neuropathologica 2019 Aug; [Epub].
Application:WB-Ce, Human, HeLa cells, Human fibroblasts.
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Characterization of the C584R variant in the mtDNA depletion syndrome gene FBXL4, reveals a novel role for FBXL4 as a regulator of mitochondrial fusion.
Sabouny R, Wong R, Lee-Glover L, Greenway SC, Sinasac DS; Care4Rare Canada, Khan A, Shutt TE.
Biochimica et Biophysica Acta. Molecular Basis of Disease 2019 Nov; 1865(11):165536.
Application:WB-Ce, Human, Human fibroblasts.
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Impaired Mitochondrial ATP Production Downregulates Wnt Signaling via ER Stress Induction.
Costa R, Peruzzo R, Bachmann M, Montà GD, Vicario M, Santinon G, Mattarei A, Moro E, Quintana-Cabrera R, Scorrano L, Zeviani M, Vallese F, Zoratti M, Paradisi C, Argenton F, Brini M, Calì T, Dupont S, Szabò I, Leanza L.
Cell Reports 2019 Aug; 28(8):1949.
Application:WB-Tr, Human, HeLa cells.
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Functional Transcriptome Analysis in ARSACS KO Cell Model Reveals a Role of Sacsin in Autophagy.
Morani F, Doccini S, Sirica R, Paterno M, Pezzini F, Ricca I, Simonati A, Delledonne M, Santorelli FM.
Scientific Reports 2019 Aug; 9(1):11878.
Application:WB-Tr, Human, SH-SY5Y cells.
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Inhibiting neddylation modification alters mitochondrial morphology and reprograms energy metabolism in cancer cells.
Zhou Q, Li H, Li Y, Tan M, Fan S, Cao C, Meng F, Zhu L, Zhao L, Guan MX, Jin H, Sun Y.
JCI Insight 2019 Feb; 4(4):121582.
Application:WB, Human, MDA-MB-231 cells.
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Acquired expression of mutant Mitofusin 2 causes progressive neurodegeneration and abnormal behavior.
Ishikawa K, Yamamoto S, Hattori S, Nishimura N, Mito T, Matsumoto H, Miyakawa T, Nakada K.
Journal of Neuroscience 2019 Feb; 39(9):1588.
Application:WB-Ti, Mouse, Mouse brains.
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Acetylation as a major determinant to microtubule-dependent autophagy: Relevance to Alzheimer's and Parkinson disease pathology.
Esteves AR, Palma AM, Gomes R, Santos D, Silva DF, Cardoso SM.
Biochimica et Biophysica Acta. Molecular Basis of Disease 2018 Dec; [Epub].
Application:WB, Human, SH-SY5Y cells.
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Administration of Enalapril Started Late in Life Attenuates Hypertrophy and Oxidative Stress Burden, Increases Mitochondrial Mass, and Modulates Mitochondrial Quality Control Signaling in the Rat Heart.
Picca A, Sirago G, Pesce V, Lezza AMS, Calvani R, Bossola M, Villani ER, Landi F, Leeuwenburgh C, Bernabei R, Carter CS, Marzetti E.
Biomolecules 2018 Dec; 8(4): E177.
Application:WB-Ti, Rat, Rat hearts.
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Mitochondrial Dynamin-Related Protein 1 (DRP1) translocation in response to cerebral glucose is impaired in a rat model of early alteration in hypothalamic glucose sensing.
Desmoulins L, Chrétien C, Paccoud R, Collins S, Cruciani-Guglielmacci C, Galinier A, Liénard F, Quinault A, Grall S, Allard C, Fenech C, Carneiro L, Mouillot T, Fournel A, Knauf C, Magnan C, Fioramonti X, Pénicaud L, Leloup C.
Molecular Metabolism 2019 Feb; 20:166.
Application:WB, Rat, Rat mediobasal hypothalamus.
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Regulation of Endoplasmic Reticulum-Mitochondria contacts by Parkin via Mfn2.
Basso V, Marchesan E, Peggion C, Chakraborty J, von Stockum S, Giacomello M, Ottolini D, Debattisti V, Caicci F, Tasca E, Pegoraro V, Angelini C, Antonini A, Bertoli A, Brini M, Ziviani E.
Pharmacological Research 2018 Sep; [Epub].
Application:WB-Tr, Mouse, MEF cells.
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Four days of bed rest increases intrinsic mitochondrial respiratory capacity in young healthy males.
Larsen S, Lundby AM, Dandanell S, Oberholzer L, Keiser S, Andersen AB, Haider T, Lundby C.
Physiological Reports 2018 Sep; 6(18):e13793.
Application:WB-Ti, Human, Muscle.
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A new mitofusin topology places the redox-regulated C terminus in the mitochondrial intermembrane space.
Mattie S, Riemer J, Wideman JG, McBride HM.
The Journal of Cell Biology 2018 Feb; 217(2):507.
Application:WB, Human, HEK 293 cells.
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The ER-Localized Transmembrane Protein EPG-3/VMP1 Regulates SERCA Activity to Control ER-Isolation Membrane Contacts for Autophagosome Formation.
Zhao YG, Chen Y, Miao G, Zhao H, Qu W, Li D, Wang Z, Liu N, Li L, Chen S, Liu P, Feng D, Zhang H.
Molecular Cell 2017 Sep; 67(6):974.
Application:WB-Tr, Monkey, COS-7 cells.
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Dietary supplementation with acetyl-l-carnitine counteracts age-related alterations of mitochondrial biogenesis, dynamics and antioxidant defenses in brain of old rats.
Nicassio L, Fracasso F, Sirago G, Musicco C, Picca A, Marzetti E, Calvani R, Cantatore P, Gadaleta MN, Pesce V.
Experimental Gerontology 2017 Aug; 98:99.
Application:WB-Ti, Rat, Rat brain.
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The thiol switch C684 in Mitofusin-2 mediates redox-induced alterations of mitochondrial shape and respiration.
Thaher O, Wolf C, Dey PN, Pouya A, Wüllner V, Tenzer S, Methner A.
Neurochemistry International 2017 May; [Epub].
Application:WB, Mouse, MEF cells.
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Physiological, biochemical, anthropometric and biomechanical influences on exercise economy in humans.
Lundby C, Montero D, Gehrig S, Anderson Hall U, Kaiser P, Boushel R, Meinild Lundby AK, Kirk N, Valdivieso P, Flück M, Secher NH, Edin F, Hein T, Madsen K.
Scandinavian Journal of Medicine & Science in Sports 2017 Feb; [Epub].
Application:WB-Ti, Human, Human skeletal muscle.
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Mitochondrial Metabolism Power SIRT2-Dependent Deficient Traffic Causing Alzheimer's-Disease Related Pathology.
Silva DF, Esteves AR, Oliveira CR, Cardoso SM.
Molecular Neurobiology 2017 Jun; 54(6):4021.
Application:WB-Ce, Human, cell lines form differentiated mild cognitive-impaired (MCI) and sporadic Alzheimer's disease (sAD)-Driven Cells.
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Mitophagy receptor FUNDC1 regulates mitochondrial dynamics and mitophagy.
Chen M, Chen Z, Wang Y, Tan Z, Zhu C, Li Y, Han Z, Chen L, Gao R, Liu L, Chen Q.
Autophagy 2016 Apr; 12(4):689.
Application:WB-Ce, Human, HeLa cells.
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DRP1-dependent apoptotic mitochondrial fission occurs independently of BAX, BAK and APAF1 to amplify cell death by BID and oxidative stress.
Oettinghaus B, D'Alonzo D, Barbieri E, Restelli LM, Savoia C, Licci M, Tolnay M, Frank S, Scorrano L.
Biochimica Et Biophysica Acta 2016 Mar; 1857(8):1267.
Application:WB-Ce, Mouse, MEF.
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Mitochondrial dysfunction in hereditary spastic paraparesis with mutations in DDHD1/SPG28.
Mignarri A, Rubegni A, Tessa A, Stefanucci S, Malandrini A, Cardaioli E, Meschini MC, Stromillo ML, Doccini S, Federico A, Santorelli FM, Dotti MT.
Amino Acids 2016 Feb; 362:287.
Application:WB-Ce, Human, Skin fibroblasts from patients with Family pedigree.
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Twenty-eight days of exposure to 3,454 m increases mitochondrial volume density in human skeletal muscle.
Jacobs RA, Lundby AM, Fenk S, Gehrig S, Siebenmann C, Fluck D, Kirk N, Hilty MP, Lundby C.
The Journal of Physiology 2016 Mar; 594(5):1151.
Application:WB-Ti, Human, Muscle.
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The Upshot of LRRK2 Inhibition to Parkinson's Disease Paradigm.
Esteves AR, G-Fernandes M, Santos D, Januario C, Cardoso SM.
Molecular Neurobiology 2015 Dec; 52(3):1804.
Application:WB-Ce, Human, Platelet mitochondria.
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Exercise training can induce cardiac autophagy at end-stage chronic conditions: Insights from a graft-versus-host-disease mouse model.
Fiuza-Luces C, Delmiro A, Soares-Miranda L, Gonzalez-Murillo A, Martinez-Palacios J, Ramirez M, Lucia A, Moran M.
Brain, Behavior, and Immunity 2014 Jul; 39:56.
Application:WB-Ti, Mouse, Ventricular.
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Regulation of miRNAs in human skeletal muscle following acute endurance exercise and short term endurance training.
Russell AP, Lamon S, Boon H, Wada S, Guller I, Brown EL, Chibalin AV, Zierath J, Snow RJ, Stepto NK, Wadley GD, Akimoto T.
The Journal of Physiology 2013 Sep; 591(18):4637.
Application:WB-Ti, Human, Skeletal muscle.
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Over-expressing mitofusin-2 in healthy mature Mammalian skeletal muscle does not alter mitochondrial bioenergetics.
Lally JS, Herbst EA, Matravadia S, Maher AC, Perry CG, Ventura-Clapier R, Holloway GP.
PLoS One 2013 Jan; 8(1):e55660.
Application:WB, Rat, Rat muscle.
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Mitofusin-2 independent juxtaposition of endoplasmic reticulum and mitochondria: an ultrastructural study.
Cosson P, Marchetti A, Ravazzola M, Orci L.
PLoS One 2012 Nov; 7(9):e46293.
Application:WB-Ce, Mouse, MEFs.
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Mitochondrial bioenergetics and dynamics interplay in complex i-deficient fibroblasts.
Moran M, Rivera H, Sanchez-Arago M, Blazquez A, Merinero B, Ugalde C, Arenas J, Cuezva JM, Martin MA.
Biochimica et Biophysica Acta 2010 May; 1802(5):443.
Application:WB-Ce, Human, Human fibroblasts.
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PGC1{alpha} relationship with skeletal muscle palmitate oxidation is not present with obesity, despite maintained ained PGC1{alpha} and PGC1{beta} protein.
Holloway GP, Perry CG, Thrush AB, Heigenhauser GJ, Dyck DJ, Bonen A, Spriet LL.
American Journal of Physiology. Endocrinology and Metabolism 2008 Mar; 294(6):E1060.
Application:WB, Human, Human skeletal muscle.
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Splice variants of mitofusin 2 shape the endoplasmic reticulum and tether it to mitochondria.
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