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Targeting mitochondrial pathology is therefore of interest. The recent development of bioactive molecules that modulate mitochondrial dynamics (fusion, fission and motility) offers a new therapeutic approach for neurodegenerative diseases with either indirect or direct mitochondrial involvement. Here, we asked: (1) Can enhanced mitochondrial fusion and motility improve secondary mitochondrial pathology in superoxide dismutase1 (SOD1) mutant amyotrophic lateral sclerosis (ALS)? And: (2) What is the impact of enhancing mitochondria fitness on in vivo manifestations of SOD1 mutant ALS? We observed that small molecule mitofusin activators corrected mitochondrial fragmentation, depolarization and dysmotility in genetically diverse ALS patient reprogrammed motor neurons and fibroblasts, and in motor neurons, sensory neurons and fibroblasts from SOD1 G93A mice. Continuous, but not intermittent, pharmacologic mitofusin activation delayed phenotype progression and lethality in SOD1 G93A mice, reducing neuron loss and improving neuromuscular connectivity. Mechanistically, mitofusin activation increased mitochondrial motility, fitness and residency within neuromuscular synapses; reduced mitochondrial reactive oxygen species production; and diminished apoptosis in SOD1 mutant neurons. These benefits were accompanied by improved mitochondrial respiratory coupling, despite characteristic SOD1 mutant ALS-associated downregulation of mitochondrial respiratory complexes. Targeting mitochondrial dysdynamism is a promising approach to alleviate pathology caused by secondary mitochondrial dysfunction in some neurodegenerative diseases.<\/jats:p>","DOI":"10.1093\/hmg\/ddac287","type":"journal-article","created":{"date-parts":[[2022,11,22]],"date-time":"2022-11-22T22:05:57Z","timestamp":1669154757000},"page":"1208-1222","source":"Crossref","is-referenced-by-count":13,"title":["Activating mitofusins interrupts mitochondrial degeneration and delays disease progression in SOD1 mutant amyotrophic lateral sclerosis"],"prefix":"10.1093","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0343-7107","authenticated-orcid":false,"given":"Xiawei","family":"Dang","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, Washington University School of Medicine , St. Louis MO USA"},{"name":"Department of Psychiatry, The First Affiliated Hospital of Xi\u2019an Jiao Tong University , Xi\u2019an, Shaanxi 710061 , China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lihong","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Washington University School of Medicine , St. Louis MO USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonietta","family":"Franco","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Washington University School of Medicine , St. Louis MO USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerald W","family":"Dorn II","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Washington University School of Medicine , St. Louis MO USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2022,11,23]]},"reference":[{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"505","DOI":"10.1038\/nrn2417","article-title":"Mitochondrial fragmentation in neurodegeneration","volume":"9","author":"Knott","year":"2008","journal-title":"Nat. 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Neurosci."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"29626","DOI":"10.1074\/jbc.M203065200","article-title":"Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice","volume":"277","author":"Mattiazzi","year":"2002","journal-title":"J. Biol. Chem."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"2720","DOI":"10.1093\/hmg\/ddm226","article-title":"Familial amyotrophic lateral sclerosis-linked SOD1 mutants perturb fast axonal transport to reduce axonal mitochondria content","volume":"16","author":"De Vos","year":"2007","journal-title":"Hum. Mol. Genet."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"1413","DOI":"10.1093\/hmg\/ddt528","article-title":"Abnormal mitochondrial transport and morphology are common pathological denominators in SOD1 and TDP43 ALS mouse models","volume":"23","author":"Magran\u00e9","year":"2014","journal-title":"Hum. Mol. 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Cell Sci."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1146\/annurev-physiol-020518-114358","article-title":"Evolving concepts of mitochondrial dynamics","volume":"81","author":"Dorn","year":"2019","journal-title":"Annu. Rev. Physiol."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1083\/jcb.200611080","article-title":"Complementation between mouse Mfn1 and Mfn2 protects mitochondrial fusion defects caused by CMT2A disease mutations","volume":"176","author":"Detmer","year":"2007","journal-title":"J. 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Neurosci."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"e0169999","DOI":"10.1371\/journal.pone.0169999","article-title":"The effect of a novel c.820C&gt;T (Arg274Trp) mutation in the mitofusin 2 gene on fibroblast metabolism and clinical manifestation in a patient","volume":"12","author":"Beresewicz","year":"2017","journal-title":"PLoS One"},{"key":"2023032011065314800_","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0028536","article-title":"Galactose enhances oxidative metabolism and reveals mitochondrial dysfunction in human primary muscle cells","volume":"6","author":"Aguer","year":"2011","journal-title":"PLoS One"},{"key":"2023032011065314800_","first-page":"1046","article-title":"Predicting mitochondrial dynamic behavior in genetically defined neurodegenerative diseases","volume":"11","author":"Dorn","year":"2022","journal-title":"Cell"},{"key":"2023032011065314800_","article-title":"Mechanisms of mitochondrial fission and fusion","volume":"5","author":"Bliek","year":"2013","journal-title":"Cold Spring Harb. Perspect. Biol."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"pdb.prot087361","DOI":"10.1101\/pdb.prot087361","article-title":"Measuring mitochondrial transmembrane potential by TMRE staining","volume":"2016","author":"Crowley","year":"2016","journal-title":"Cold Spring Harb Protoc"},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"7033","DOI":"10.1021\/acs.jmedchem.0c00366","article-title":"Discovery of 6-phenylhexanamide derivatives as potent stereoselective mitofusin activators for the treatment of mitochondrial diseases","volume":"63","author":"Dang","year":"2020","journal-title":"J. Med. Chem."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1126\/science.aao1785","article-title":"MFN2 agonists reverse mitochondrial defects in preclinical models of Charcot-Marie-Tooth disease type 2A","volume":"360","author":"Rocha","year":"2018","journal-title":"Science"},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"332","DOI":"10.1016\/j.stem.2017.08.002","article-title":"MicroRNAs induce a permissive chromatin environment that enables neuronal subtype-specific reprogramming of adult human fibroblasts","volume":"21","author":"Abernathy","year":"2017","journal-title":"Cell Stem Cell"},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"12506","DOI":"10.1021\/acs.jmedchem.1c00163","article-title":"Pharmacophore-based design of phenyl-[hydroxycyclohexyl] cycloalkyl-carboxamide mitofusin activators with improved neuronal activity","volume":"64","author":"Dang","year":"2021","journal-title":"J. Med. Chem."},{"key":"2023032011065314800_","article-title":"A simple composite phenotype scoring system for evaluating mouse models of cerebellar ataxia","volume":"39","author":"Guyenet","year":"2010","journal-title":"J. Vis. Exp."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"1488","DOI":"10.1002\/1097-4598(200010)23:10<1488::AID-MUS4>3.0.CO;2-E","article-title":"Electrodiagnostic studies in amyotrophic lateral sclerosis and other motor neuron disorders","volume":"23","author":"Daube","year":"2000","journal-title":"Muscle Nerve"},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.jns.2005.02.006","article-title":"Background and gender effects on survival in the TgN(SOD1-G93A)1Gur mouse model of ALS","volume":"236","author":"Heiman-Patterson","year":"2005","journal-title":"J. Neurol. Sci."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1097\/nen.0b013e31802c396b","article-title":"Mitochondrial alterations in the spinal cord of patients with sporadic amyotrophic lateral sclerosis","volume":"66","author":"Sasaki","year":"2007","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1016\/j.neuron.2004.06.021","article-title":"Amyotrophic lateral sclerosis-associated SOD1 mutant proteins bind and aggregate with Bcl-2 in spinal cord mitochondria","volume":"43","author":"Pasinelli","year":"2004","journal-title":"Neuron"},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"2974","DOI":"10.1093\/hmg\/ddq202","article-title":"ALS-linked mutant SOD1 damages mitochondria by promoting conformational changes in Bcl-2","volume":"19","author":"Pedrini","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"179","DOI":"10.3389\/fncel.2014.00179","article-title":"CIIA prevents SOD1(G93A)-induced cytotoxicity by blocking ASK1-mediated signaling","volume":"8","author":"Lee","year":"2014","journal-title":"Front. Cell. Neurosci."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"3947","DOI":"10.1093\/hmg\/ddt249","article-title":"Mitochondrial damage revealed by immunoselection for ALS-linked misfolded SOD1","volume":"22","author":"Pickles","year":"2013","journal-title":"Hum. Mol. Genet."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"749","DOI":"10.1111\/j.1474-9726.2011.00709.x","article-title":"Role of superoxide-nitric oxide interactions in the accelerated age-related loss of muscle mass in mice lacking Cu,Zn superoxide dismutase","volume":"10","author":"Sakellariou","year":"2011","journal-title":"Aging Cell"},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"6569","DOI":"10.1523\/JNEUROSCI.21-17-06569.2001","article-title":"Recruitment of the mitochondrial-dependent apoptotic pathway in amyotrophic lateral sclerosis","volume":"21","author":"Gu\u00e9gan","year":"2001","journal-title":"J. Neurosci."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1523\/JNEUROSCI.3829-04.2005","article-title":"Cytochrome c association with the inner mitochondrial membrane is impaired in the CNS of G93A-SOD1 mice","volume":"25","author":"Kirkinezos","year":"2005","journal-title":"J. Neurosci."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","DOI":"10.1016\/j.xpro.2020.100245","article-title":"Measurement of mitochondrial respiration in adherent cells by Seahorse XF96 Cell Mito Stress Test","volume":"2","author":"Gu","year":"2021","journal-title":"STAR Protoc."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"599","DOI":"10.1016\/j.nbd.2006.01.001","article-title":"Bioenergetic abnormalities in discrete cerebral motor pathways presage spinal cord pathology in the G93A SOD1 mouse model of ALS","volume":"22","author":"Browne","year":"2006","journal-title":"Neurobiol. Dis."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1016\/j.ceca.2011.02.004","article-title":"G37R SOD1 mutant alters mitochondrial complex I activity, Ca(2+) uptake and ATP production","volume":"49","author":"Coussee","year":"2011","journal-title":"Cell Calcium"},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"548","DOI":"10.1016\/j.cell.2007.06.026","article-title":"Mitochondrial fusion protects against neurodegeneration in the cerebellum","volume":"130","author":"Chen","year":"2007","journal-title":"Cell"},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"1327","DOI":"10.1161\/CIRCRESAHA.111.258723","article-title":"Mitochondrial fusion is essential for organelle function and cardiac homeostasis","volume":"109","author":"Chen","year":"2011","journal-title":"Circ. Res."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0044296","article-title":"Two rare human mitofusin 2 mutations alter mitochondrial dynamics and induce retinal and cardiac pathology in Drosophila","volume":"7","author":"Eschenbacher","year":"2012","journal-title":"PLoS One"},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"734","DOI":"10.1126\/science.1241359","article-title":"Mitochondrial fusion directs cardiomyocyte differentiation via calcineurin and Notch signaling","volume":"342","author":"Kasahara","year":"2013","journal-title":"Science"},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1016\/j.cmet.2014.12.011","article-title":"Mitochondrial fission and fusion factors reciprocally orchestrate mitophagic culling in mouse hearts and cultured fibroblasts","volume":"21","author":"Song","year":"2015","journal-title":"Cell Metab."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"872","DOI":"10.1016\/j.cmet.2017.09.023","article-title":"Abrogating mitochondrial dynamics in mouse hearts accelerates mitochondrial senescence","volume":"26","author":"Song","year":"2017","journal-title":"Cell Metab."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","DOI":"10.1172\/JCI147307","article-title":"Restoring mitofusin balance prevents axonal degeneration in a Charcot-Marie-Tooth type 2A model","volume":"131","author":"Zhou","year":"2021","journal-title":"J. Clin. Invest."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"449","DOI":"10.1038\/ng1341","article-title":"Mutations in the mitochondrial GTPase mitofusin 2 cause Charcot-Marie-Tooth neuropathy type 2A","volume":"36","author":"Zuchner","year":"2004","journal-title":"Nat. Genet."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"2093","DOI":"10.1093\/brain\/awl126","article-title":"MFN2 mutation distribution and genotype\/phenotype correlation in Charcot-Marie-Tooth type 2","volume":"129","author":"Verhoeven","year":"2006","journal-title":"Brain"},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"1690","DOI":"10.1212\/WNL.0b013e31821a441e","article-title":"MFN2 mutations cause severe phenotypes in most patients with CMT2A","volume":"76","author":"Feely","year":"2011","journal-title":"Neurology"},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"1036","DOI":"10.1001\/jamaneurol.2014.629","article-title":"Charcot-Marie-Tooth disease type 2A, from typical to rare phenotypic and genotypic features","volume":"71","author":"Bombelli","year":"2014","journal-title":"JAMA Neurol."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"655","DOI":"10.3390\/chemistry4030047","article-title":"Piperine derivatives enhance fusion and axonal transport of mitochondria by activating mitofusins","volume":"4","author":"Zhang","year":"2022","journal-title":"Chemistry"},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1038\/nature20156","article-title":"Correcting mitochondrial fusion by manipulating mitofusin conformations","volume":"540","author":"Franco","year":"2016","journal-title":"Nature"},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"3775","DOI":"10.1038\/s41467-022-31324-1","article-title":"Modulating mitofusins to control mitochondrial function and signaling","volume":"13","author":"Zacharioudakis","year":"2022","journal-title":"Nat. Commun."},{"key":"2023032011065314800_","article-title":"Reciprocal regulation of mitofusin 2-mediated mitophagy and mitochondrial fusion by different PINK1 phosphorylation events","volume":"10","author":"Li","year":"2022","journal-title":"Front. Cell Dev. Biol."},{"key":"2023032011065314800_","first-page":"3","article-title":"Mitochondrial electron transport chain, ROS generation and uncoupling (Review)","volume":"44","author":"Zhao","year":"2019","journal-title":"Int. J. Mol. Med."},{"key":"2023032011065314800_","doi-asserted-by":"crossref","first-page":"16763","DOI":"10.1038\/srep16763","article-title":"High yield extraction of pure spinal motor neurons, astrocytes and microglia from single embryo and adult mouse spinal cord","volume":"5","author":"Beaudet","year":"2015","journal-title":"Sci. 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