{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T16:42:35Z","timestamp":1790959355240,"version":"4.1.0"},"reference-count":174,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2017,4,5]],"date-time":"2017-04-05T00:00:00Z","timestamp":1491350400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Antioxidants"],"abstract":"<jats:p>Neurodegenerative diseases are incurable and devastating neurological disorders characterized by the progressive loss of the structure and function of neurons in the central nervous system or peripheral nervous system. Mitochondria, organelles found in most eukaryotic cells, are essential for neuronal survival and are involved in a number of neuronal functions. Mitochondrial dysfunction has long been demonstrated as a common prominent early pathological feature of a variety of common neurodegenerative diseases, including Alzheimer\u2019s disease (AD), Parkinson\u2019s disease (PD), amyotrophic lateral sclerosis (ALS), and Huntington\u2019s disease (HD). Mitochondria are highly dynamic organelles that undergo continuous fusion, fission, and transport, the processes of which not only control mitochondrial morphology and number but also regulate mitochondrial function and location. The importance of mitochondrial dynamics in the pathogenesis of neurodegenerative diseases has been increasingly unraveled after the identi\ufb01cation of several key fusion and fission regulators such as Drp1, OPA1, and mitofusins. In this review, after a brief discussion of molecular mechanisms regulating mitochondrial fusion, fission, distribution, and trafficking, as well as the important role of mitochondrial dynamics for neuronal function, we review previous and the most recent studies about mitochondrial dynamic abnormalities observed in various major neurodegenerative diseases and discuss the possibility of targeting mitochondrial dynamics as a likely novel therapeutic strategy for neurodegenerative diseases.<\/jats:p>","DOI":"10.3390\/antiox6020025","type":"journal-article","created":{"date-parts":[[2017,4,5]],"date-time":"2017-04-05T10:33:01Z","timestamp":1491388381000},"page":"25","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":209,"title":["Abnormalities of Mitochondrial Dynamics in Neurodegenerative Diseases"],"prefix":"10.3390","volume":"6","author":[{"given":"Ju","family":"Gao","sequence":"first","affiliation":[{"name":"Departments of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luwen","family":"Wang","sequence":"additional","affiliation":[{"name":"Departments of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jingyi","family":"Liu","sequence":"additional","affiliation":[{"name":"Departments of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fei","family":"Xie","sequence":"additional","affiliation":[{"name":"Departments of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bo","family":"Su","sequence":"additional","affiliation":[{"name":"Department of Neurobiology, Shandong University, Jinan 250012, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xinglong","family":"Wang","sequence":"additional","affiliation":[{"name":"Departments of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2017,4,5]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1577","DOI":"10.1101\/gad.1658508","article-title":"Mitochondrial dynamics and apoptosis","volume":"22","author":"Suen","year":"2008","journal-title":"Genes Dev."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"870","DOI":"10.1038\/nrm2275","article-title":"Functions and dysfunctions of mitochondrial dynamics","volume":"8","author":"Detmer","year":"2007","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"R169","DOI":"10.1093\/hmg\/ddp326","article-title":"Mitochondrial dynamics\u2014Fusion, fission, movement, and mitophagy\u2014In neurodegenerative diseases","volume":"18","author":"Chen","year":"2009","journal-title":"Hum. Mol. Genet."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1111\/j.1471-4159.2009.05867.x","article-title":"The role of abnormal mitochondrial dynamics in the pathogenesis of Alzheimer\u2019s disease","volume":"109","author":"Wang","year":"2009","journal-title":"J. Neurochem."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"C641","DOI":"10.1152\/ajpcell.00222.2006","article-title":"Mitochondria and neuronal activity","volume":"292","author":"Kann","year":"2007","journal-title":"Am. J. Physiol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"873","DOI":"10.1016\/j.cell.2004.11.003","article-title":"The importance of dendritic mitochondria in the morphogenesis and plasticity of spines and synapses","volume":"119","author":"Li","year":"2004","journal-title":"Cell"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1038\/nrn3156","article-title":"Mitochondrial transport in neurons: Impact on synaptic homeostasis and neurodegeneration","volume":"13","author":"Sheng","year":"2012","journal-title":"Nat. Rev. Neurosci."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1038\/nature05292","article-title":"Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases","volume":"443","author":"Lin","year":"2006","journal-title":"Nature"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1159\/000017165","article-title":"Cerebrometabolic aspects of delirium in relationship to dementia","volume":"10","author":"Blass","year":"1999","journal-title":"Dement. Geriatr. Cogn. Disord."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"838","DOI":"10.1242\/jcs.03381","article-title":"Mitochondrial bioenergetics and structural network organization","volume":"120","author":"Benard","year":"2007","journal-title":"J. Cell Sci."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"22704","DOI":"10.1074\/jbc.M312366200","article-title":"Ca2+ homeostasis during mitochondrial fragmentation and perinuclear clustering induced by hFis1","volume":"279","author":"Frieden","year":"2004","journal-title":"J. Biol. Chem."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1016\/j.molcel.2004.09.026","article-title":"Drp-1-dependent division of the mitochondrial network blocks intraorganellar Ca2+ waves and protects against Ca2+-mediated apoptosis","volume":"16","author":"Szabadkai","year":"2004","journal-title":"Mol. Cell"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1016\/S1534-5807(01)00055-7","article-title":"The role of dynamin-related protein 1, a mediator of mitochondrial fission, in apoptosis","volume":"1","author":"Frank","year":"2001","journal-title":"Dev. Cell"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"52726","DOI":"10.1074\/jbc.M408910200","article-title":"Fzo1, a protein involved in mitochondrial fusion, inhibits apoptosis","volume":"279","author":"Sugioka","year":"2004","journal-title":"J. Biol. Chem."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"7743","DOI":"10.1074\/jbc.C200677200","article-title":"Loss of OPA1 perturbates the mitochondrial inner membrane structure and integrity, leading to cytochrome c release and apoptosis","volume":"278","author":"Olichon","year":"2003","journal-title":"J. Biol. Chem."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"2653","DOI":"10.1073\/pnas.0511154103","article-title":"Increased production of reactive oxygen species in hyperglycemic conditions requires dynamic change of mitochondrial morphology","volume":"103","author":"Yu","year":"2006","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1016\/j.tcb.2012.10.006","article-title":"Mitochondrial dynamics in neurodegeneration","volume":"23","author":"Itoh","year":"2013","journal-title":"Trends Cell Biol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1038\/nrneurol.2014.228","article-title":"Disturbed mitochondrial dynamics and neurodegenerative disorders","volume":"11","author":"Burte","year":"2015","journal-title":"Nat. Rev. Neurol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"2245","DOI":"10.1091\/mbc.12.8.2245","article-title":"Dynamin-related protein Drp1 is required for mitochondrial division in mammalian cells","volume":"12","author":"Smirnova","year":"2001","journal-title":"Mol. Biol. Cell"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1141","DOI":"10.1083\/jcb.201007152","article-title":"Mff is an essential factor for mitochondrial recruitment of Drp1 during mitochondrial fission in mammalian cells","volume":"191","author":"Otera","year":"2010","journal-title":"J. Cell Biol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"565","DOI":"10.1038\/embor.2011.54","article-title":"MiD49 and MiD51, new components of the mitochondrial fission machinery","volume":"12","author":"Palmer","year":"2011","journal-title":"Embo Rep."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1091\/mbc.e12-10-0721","article-title":"Fis1, Mff, MiD49, and MiD51 mediate Drp1 recruitment in mitochondrial fission","volume":"24","author":"Loson","year":"2013","journal-title":"Mol. Biol. Cell"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"36373","DOI":"10.1074\/jbc.M303758200","article-title":"hFis1, a novel component of the mammalian mitochondrial fission machinery","volume":"278","author":"James","year":"2003","journal-title":"J. Biol. Chem."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1038\/nature20555","article-title":"Multiple dynamin family members collaborate to drive mitochondrial division","volume":"540","author":"Lee","year":"2016","journal-title":"Nature"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"358","DOI":"10.1126\/science.1207385","article-title":"ER tubules mark sites of mitochondrial division","volume":"334","author":"Friedman","year":"2011","journal-title":"Science"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"464","DOI":"10.1126\/science.1228360","article-title":"An actin-dependent step in mitochondrial fission mediated by the ER-associated formin INF2","volume":"339","author":"Korobova","year":"2013","journal-title":"Science"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1146\/annurev.cellbio.22.010305.104638","article-title":"Mitochondrial fusion and fission in mammals","volume":"22","author":"Chan","year":"2006","journal-title":"Annu. Rev. Cell Dev. Biol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"1138","DOI":"10.1016\/j.bbadis.2008.11.011","article-title":"The molecular mechanism and cellular functions of mitochondrial division","volume":"1792","author":"Lackner","year":"2009","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"17783","DOI":"10.1074\/jbc.M901902200","article-title":"Translocation of SenP5 from the nucleoli to the mitochondria modulates Drp1-dependent fission during mitosis","volume":"284","author":"Zunino","year":"2009","journal-title":"J. Biol. Chem."},{"key":"ref_30","doi-asserted-by":"crossref","unstructured":"Merrill, R.A., Dagda, R.K., Dickey, A.S., Cribbs, J.T., Green, S.H., Usachev, Y.M., and Strack, S. (2011). Mechanism of neuroprotective mitochondrial remodeling by PKA\/AKAP1. PLoS Biol., 9.","DOI":"10.1371\/journal.pbio.1000612"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1038\/nm.3983","article-title":"Parkinson\u2019s disease-associated mutant VPS35 causes mitochondrial dysfunction by recycling DLP1 complexes","volume":"22","author":"Wang","year":"2016","journal-title":"Nat. Med."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"867","DOI":"10.1242\/jcs.114.5.867","article-title":"Control of mitochondrial morphology by a human mitofusin","volume":"114","author":"Santel","year":"2001","journal-title":"J. Cell Sci."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"6535","DOI":"10.1242\/jcs.01565","article-title":"Mitofusin 1 and 2 play distinct roles in mitochondrial fusion reactions via GTPase activity","volume":"117","author":"Ishihara","year":"2004","journal-title":"J. Cell Sci."},{"key":"ref_34","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":"ref_35","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1007\/s00109-014-1237-z","article-title":"Role of mitochondrial lipids in guiding fission and fusion","volume":"93","author":"Frohman","year":"2015","journal-title":"J. Mol. Med. Berl."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"1255","DOI":"10.1038\/ncb1487","article-title":"A common lipid links Mfn-mediated mitochondrial fusion and SNARE-regulated exocytosis","volume":"8","author":"Choi","year":"2006","journal-title":"Nat. Cell Biol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/j.molcel.2015.11.017","article-title":"Mitoguardin regulates mitochondrial fusion through mitopld and is required for neuronal homeostasis","volume":"61","author":"Zhang","year":"2016","journal-title":"Mol. Cell"},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"889","DOI":"10.1016\/j.cell.2010.08.017","article-title":"Membrane remodeling induced by the dynamin-related protein Drp1 stimulates Bax oligomerization","volume":"142","author":"Montessuit","year":"2010","journal-title":"Cell"},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"1905","DOI":"10.1091\/mbc.e14-02-0728","article-title":"A dimeric equilibrium intermediate nucleates Drp1 reassembly on mitochondrial membranes for fission","volume":"25","author":"Macdonald","year":"2014","journal-title":"Mol. Biol. Cell"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"1668","DOI":"10.1111\/j.1600-0854.2007.00644.x","article-title":"Moving mitochondria: Establishing distribution of an essential organelle","volume":"8","author":"Frederick","year":"2007","journal-title":"Traffic"},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"1087","DOI":"10.1083\/jcb.201312123","article-title":"Mitochondrial trafficking and anchoring in neurons: New insight and implications","volume":"204","author":"Sheng","year":"2014","journal-title":"J. Cell Biol."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1016\/j.neuron.2005.06.027","article-title":"The GTPase dmiro is required for axonal transport of mitochondria to drosophila synapses","volume":"47","author":"Guo","year":"2005","journal-title":"Neuron"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"545","DOI":"10.1083\/jcb.200601067","article-title":"Axonal transport of mitochondria requires milton to recruit kinesin heavy chain and is light chain independent","volume":"173","author":"Glater","year":"2006","journal-title":"J. Cell Biol."},{"key":"ref_44","first-page":"4549","article-title":"Novel roles for actin in mitochondrial fission","volume":"127","author":"Hatch","year":"2014","journal-title":"J. Cell Sci."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1083\/jcb.200709099","article-title":"The class V myosin motor protein, myo2, plays a major role in mitochondrial motility in Saccharomyces cerevisiae","volume":"181","author":"Altmann","year":"2008","journal-title":"J. Cell Biol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"3162","DOI":"10.1073\/pnas.051494698","article-title":"Arp2\/3 complex and actin dynamics are required for actin-based mitochondrial motility in yeast","volume":"98","author":"Boldogh","year":"2001","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"18792","DOI":"10.1074\/jbc.M400920200","article-title":"Loss of the intermembrane space protein Mgm1\/OPA1 induces swelling and localized constrictions along the lengths of mitochondria","volume":"279","author":"Griparic","year":"2004","journal-title":"J. Biol. Chem."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"3291","DOI":"10.1093\/hmg\/ddn225","article-title":"A novel deletion in the GTPase domain of OPA1 causes defects in mitochondrial morphology and distribution, but not in function","volume":"17","author":"Spinazzi","year":"2008","journal-title":"Hum. Mol. Genet."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1038\/nm.2313","article-title":"Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity","volume":"17","author":"Song","year":"2011","journal-title":"Nat. Med."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1016\/j.mcn.2015.12.006","article-title":"Mitochondrial fission protein Drp1 regulates mitochondrial transport and dendritic arborization in cerebellar Purkinje cells","volume":"71","author":"Fukumitsu","year":"2016","journal-title":"Mol. Cell Neurosci."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"4232","DOI":"10.1523\/JNEUROSCI.6248-09.2010","article-title":"Mitofusin 2 is necessary for transport of axonal mitochondria and interacts with the Miro\/Milton complex","volume":"30","author":"Misko","year":"2010","journal-title":"J. Neurosci."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"9633","DOI":"10.1073\/pnas.1404651111","article-title":"Transcellular degradation of axonal mitochondria","volume":"111","author":"Davis","year":"2014","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"367","DOI":"10.1038\/nature21362","article-title":"Elegans neurons jettison protein aggregates and mitochondria under neurotoxic stress","volume":"542","author":"Melentijevic","year":"2017","journal-title":"Nature"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1038\/nature18928","article-title":"Transfer of mitochondria from astrocytes to neurons after stroke","volume":"535","author":"Hayakawa","year":"2016","journal-title":"Nature"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1093\/jb\/mvi169","article-title":"PDIP38 associates with proteins constituting the mitochondrial DNA nucleoid","volume":"138","author":"Cheng","year":"2005","journal-title":"J. Biochem."},{"key":"ref_56","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":"ref_57","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1038\/sj.emboj.7601963","article-title":"Fission and selective fusion govern mitochondrial segregation and elimination by autophagy","volume":"27","author":"Twig","year":"2008","journal-title":"EMBO J."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"1767","DOI":"10.1093\/nar\/27.8.1767","article-title":"Animal mitochondrial genomes","volume":"27","author":"Boore","year":"1999","journal-title":"Nucleic Acids Res."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"40392","DOI":"10.1074\/jbc.M404316200","article-title":"The modulation in subunits e and g amounts of yeast ATP synthase modifies mitochondrial cristae morphology","volume":"279","author":"Arselin","year":"2004","journal-title":"J. Biol. Chem."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1083\/jcb.30.2.269","article-title":"Ultrastructural bases for metabolically linked mechanical activity in mitochondria. I. Reversible ultrastructural changes with change in metabolic steady state in isolated liver mitochondria","volume":"30","author":"Hackenbrock","year":"1966","journal-title":"J. Cell Biol."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"R551","DOI":"10.1016\/j.cub.2006.06.054","article-title":"Mitochondria: More than just a powerhouse","volume":"16","author":"McBride","year":"2006","journal-title":"Curr. Biol."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"8057","DOI":"10.1073\/pnas.88.18.8057","article-title":"Dynamics, structure, and function are coupled in the mitochondrial matrix","volume":"88","author":"Scalettar","year":"1991","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"1313","DOI":"10.1089\/ars.2007.2000","article-title":"Ultrastructure of the mitochondrion and its bearing on function and bioenergetics","volume":"10","author":"Benard","year":"2008","journal-title":"Antioxid. Redox Signal."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"12920","DOI":"10.1073\/pnas.1107332108","article-title":"Pink1 regulates the oxidative phosphorylation machinery via mitochondrial fission","volume":"108","author":"Liu","year":"2011","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1016\/j.cell.2013.08.032","article-title":"Mitochondrial cristae shape determines respiratory chain supercomplexes assembly and respiratory efficiency","volume":"155","author":"Cogliati","year":"2013","journal-title":"Cell"},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"958","DOI":"10.1038\/ncb1907","article-title":"Mitochondrial fission factor Drp1 is essential for embryonic development and synapse formation in mice","volume":"11","author":"Ishihara","year":"2009","journal-title":"Nat. Cell Biol."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1083\/jcb.200903065","article-title":"The dynamin-related GTPase Drp1 is required for embryonic and brain development in mice","volume":"186","author":"Wakabayashi","year":"2009","journal-title":"J. Cell Biol."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"E3631","DOI":"10.1073\/pnas.1402449111","article-title":"Loss of Miro1-directed mitochondrial movement results in a novel murine model for neuron disease","volume":"111","author":"Nguyen","year":"2014","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"541","DOI":"10.1016\/j.neuron.2009.01.030","article-title":"Miro1 is a calcium sensor for glutamate receptor-dependent localization of mitochondria at synapses","volume":"61","author":"Macaskill","year":"2009","journal-title":"Neuron"},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1016\/j.mcn.2008.10.016","article-title":"GTPase dependent recruitment of Grif-1 by Miro1 regulates mitochondrial trafficking in hippocampal neurons","volume":"40","author":"MacAskill","year":"2009","journal-title":"Mol. Cell Neurosci."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1177\/1073858407307354","article-title":"Mitochondrial dynamics and peripheral neuropathy","volume":"14","author":"Baloh","year":"2008","journal-title":"Neuroscientist"},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"2639","DOI":"10.1093\/hmg\/ddt659","article-title":"Neuromuscular junction maturation defects precede impaired lower motor neuron connectivity in Charcot-Marie-Tooth type 2D mice","volume":"23","author":"Sleigh","year":"2014","journal-title":"Hum. Mol. Genet."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1038\/79936","article-title":"Nuclear gene OPA1, encoding a mitochondrial dynamin-related protein, is mutated in dominant optic atrophy","volume":"26","author":"Delettre","year":"2000","journal-title":"Nat. Genet."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1038\/79944","article-title":"OPA1, encoding a dynamin-related GTPase, is mutated in autosomal dominant optic atrophy linked to chromosome 3q28","volume":"26","author":"Alexander","year":"2000","journal-title":"Nat. Genet."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/S0074-7742(08)60607-8","article-title":"Alzheimer disease","volume":"42","author":"Smith","year":"1998","journal-title":"Int. Rev. Neurobiol."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1002\/jnr.10389","article-title":"Role of mitochondrial dysfunction in Alzheimer\u2019s disease","volume":"70","author":"Castellani","year":"2002","journal-title":"J. Neurosci. Res."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"3017","DOI":"10.1523\/JNEUROSCI.21-09-03017.2001","article-title":"Mitochondrial abnormalities in Alzheimer\u2019s disease","volume":"21","author":"Hirai","year":"2001","journal-title":"J. Neurosci."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"9090","DOI":"10.1523\/JNEUROSCI.1357-09.2009","article-title":"Impaired balance of mitochondrial fission and fusion in Alzheimer\u2019s disease","volume":"29","author":"Wang","year":"2009","journal-title":"J. Neurosci."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"18754","DOI":"10.1074\/jbc.M808908200","article-title":"Caspase-cleaved tau expression induces mitochondrial dysfunction in immortalized cortical neurons: Implications for the pathogenesis of Alzheimer disease","volume":"284","author":"Quintanilla","year":"2009","journal-title":"J. Biol. Chem."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"2538","DOI":"10.1093\/hmg\/dds072","article-title":"Abnormal interaction between the mitochondrial fission protein Drp1 and hyperphosphorylated tau in Alzheimers disease neurons: Implications for mitochondrial dysfunction and neuronal damage","volume":"21","author":"Manczak","year":"2012","journal-title":"Hum. Mol. Genet."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"618","DOI":"10.1016\/j.neuron.2012.06.026","article-title":"Tau promotes neurodegeneration via Drp1 mislocalization in vivo","volume":"75","author":"DuBoff","year":"2012","journal-title":"Neuron"},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"24756","DOI":"10.1038\/srep24756","article-title":"Human wild-type full-length tau accumulation disrupts mitochondrial dynamics and the functions via increasing mitofusins","volume":"6","author":"Li","year":"2016","journal-title":"Sci. Rep."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"19318","DOI":"10.1073\/pnas.0804871105","article-title":"Amyloid-beta overproduction causes abnormal mitochondrial dynamics via differential modulation of mitochondrial fission\/fusion proteins","volume":"105","author":"Wang","year":"2008","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1038\/nm0296-224","article-title":"Alzheimer-associated presenilins 1 and 2: Neuronal expression in brain and localization to intracellular membranes in mammalian cells","volume":"2","author":"Kovacs","year":"1996","journal-title":"Nat. Med."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"1030","DOI":"10.1046\/j.1471-4159.1999.0721030.x","article-title":"Altered calcium homeostasis and mitochondrial dysfunction in cortical synaptic compartments of presenilin-1 mutant mice","volume":"72","author":"Begley","year":"1999","journal-title":"J. Neurochem."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"2749","DOI":"10.1111\/j.1460-9568.2004.03732.x","article-title":"Altered morphological and electrophysiological properties of Cajal-Retzius cells in cerebral cortex of embryonic Presenilin-1 knockout mice","volume":"20","author":"Kilb","year":"2004","journal-title":"Eur. J. Neurosci."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"891","DOI":"10.1002\/jnr.20990","article-title":"Differential role of presenilin-1 and -2 on mitochondrial membrane potential and oxygen consumption in mouse embryonic fibroblasts","volume":"84","author":"Behbahani","year":"2006","journal-title":"J. Neurosci. Res."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1016\/j.neulet.2005.10.088","article-title":"Calcium oscillations in type-1 astrocytes, the effect of a presenilin 1 (PS1) mutation","volume":"395","author":"Johnston","year":"2006","journal-title":"Neurosci. Lett."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"409","DOI":"10.3233\/JAD-2001-3408","article-title":"Ultrastructure evidence of necrotic neural cell death in familial Alzheimer\u2019s disease brains bearing presenilin-1 E280A mutation","volume":"3","author":"Arroyave","year":"2001","journal-title":"J. Alzheimers Dis."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"14670","DOI":"10.1073\/pnas.0903563106","article-title":"Mitochondrial bioenergetic deficit precedes Alzheimer\u2019s pathology in female mouse model of Alzheimer\u2019s disease","volume":"106","author":"Yao","year":"2009","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"18670","DOI":"10.1073\/pnas.1006586107","article-title":"Early deficits in synaptic mitochondria in an Alzheimer\u2019s disease mouse model","volume":"107","author":"Du","year":"2010","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"4881","DOI":"10.1093\/hmg\/ddw312","article-title":"Reduced dynamin-related protein 1 protects against phosphorylated Tau-induced mitochondrial dysfunction and synaptic damage in alzheimer\u2019s disease","volume":"25","author":"Kandimalla","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"ref_93","first-page":"5148","article-title":"Protective effects of reduced dynamin-related protein 1 against amyloid beta-induced mitochondrial dysfunction and synaptic damage in Alzheimer\u2019s disease","volume":"25","author":"Manczak","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"1282","DOI":"10.1126\/science.1105681","article-title":"Axonopathy and transport deficits early in the pathogenesis of Alzheimer\u2019s disease","volume":"307","author":"Stokin","year":"2005","journal-title":"Science"},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"10480","DOI":"10.1523\/JNEUROSCI.3231-06.2006","article-title":"Acute impairment of mitochondrial trafficking by beta-amyloid peptides in hippocampal neurons","volume":"26","author":"Rui","year":"2006","journal-title":"J. Neurosci."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"2430","DOI":"10.1523\/JNEUROSCI.5927-11.2012","article-title":"Regulation of mitochondrial transport and inter-microtubule spacing by tau phosphorylation at the sites hyperphosphorylated in Alzheimer\u2019s disease","volume":"32","author":"Shahpasand","year":"2012","journal-title":"J. Neurosci."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.nbd.2015.10.007","article-title":"Reduced number of axonal mitochondria and tau hypophosphorylation in mouse p301l tau knockin neurons","volume":"85","author":"Pooler","year":"2016","journal-title":"Neurobiol. Dis."},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"1972","DOI":"10.1016\/j.biocel.2009.05.013","article-title":"Ca2+-dependent regulation of mitochondrial dynamics by the Miro-Milton complex","volume":"41","author":"Liu","year":"2009","journal-title":"Int. J. Biochem. Cell Biol."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"13145","DOI":"10.1073\/pnas.0806192105","article-title":"The amyloid beta-peptide is imported into mitochondria via the TOM import machinery and localized to mitochondrial cristae","volume":"105","author":"Petersen","year":"2008","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"7916","DOI":"10.1073\/pnas.1300677110","article-title":"Modulation of the endoplasmic reticulum-mitochondria interface in Alzheimer\u2019s disease and related models","volume":"110","author":"Hedskog","year":"2013","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"357","DOI":"10.4161\/cib.4.3.15160","article-title":"Presenilin-2 modulation of ER-mitochondria interactions: FAD mutations, mechanisms and pathological consequences","volume":"4","author":"Zampese","year":"2011","journal-title":"Commun. Integr. Biol."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"2938","DOI":"10.1093\/hmg\/ddv056","article-title":"Parkin-mediated mitophagy in mutant hAPP neurons and Alzheimer\u2019s disease patient brains","volume":"24","author":"Ye","year":"2015","journal-title":"Hum. Mol. Genet."},{"key":"ref_103","first-page":"385","article-title":"Autophagy in Alzheimer\u2019s disease","volume":"26","author":"Masliah","year":"2015","journal-title":"Rev. Neurosci."},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"259","DOI":"10.31887\/DCNS.2004.6.3\/galexander","article-title":"Biology of Parkinson\u2019s disease: Pathogenesis and pathophysiology of a multisystem neurodegenerative disorder","volume":"6","author":"Alexander","year":"2004","journal-title":"Dialogues Clin. Neurosci."},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"979","DOI":"10.1126\/science.6823561","article-title":"Chronic parkinsonism in humans due to a product of meperidine-analog synthesis","volume":"219","author":"Langston","year":"1983","journal-title":"Science"},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1002\/syn.890180105","article-title":"MPTP-induced oxidative stress and neurotoxicity are age-dependent: Evidence from measures of reactive oxygen species and striatal dopamine levels","volume":"18","author":"Ali","year":"1994","journal-title":"Synapse"},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"807","DOI":"10.1111\/j.1474-9726.2011.00721.x","article-title":"DLP1-dependent mitochondrial fragmentation mediates 1-methyl-4-phenylpyridinium toxicity in neurons: Implications for Parkinson\u2019s disease","volume":"10","author":"Wang","year":"2011","journal-title":"Aging Cell"},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"4545","DOI":"10.1093\/hmg\/ddt301","article-title":"Inhibition of excessive mitochondrial fission reduced aberrant autophagy and neuronal damage caused by LRRK2 G2019S mutation","volume":"22","author":"Su","year":"2013","journal-title":"Hum. Mol. Genet."},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"2017","DOI":"10.1212\/WNL.0b013e3181ff9685","article-title":"Mitochondrial impairment in patients with parkinson disease with the G2019S mutation in LRRK2","volume":"75","author":"Mortiboys","year":"2010","journal-title":"Neurology"},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"4201","DOI":"10.1093\/hmg\/dds244","article-title":"G2019S leucine-rich repeat kinase 2 causes uncoupling protein-mediated mitochondrial depolarization","volume":"21","author":"Papkovskaia","year":"2012","journal-title":"Hum. Mol. Genet."},{"key":"ref_111","doi-asserted-by":"crossref","unstructured":"Haylett, W., Swart, C., van der Westhuizen, F., van Dyk, H., van der Merwe, L., van der Merwe, C., Loos, B., Carr, J., Kinnear, C., and Bardien, S. (2016). Altered mitochondrial respiration and other features of mitochondrial function in Parkin-mutant fibroblasts from Parkinson\u2019s disease patients. Parkinsons Dis.","DOI":"10.1155\/2016\/1819209"},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"9747","DOI":"10.1073\/pnas.0911175107","article-title":"DJ-1 is critical for mitochondrial function and rescues PINK1 loss of function","volume":"107","author":"Hao","year":"2010","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1093\/hmg\/ddq430","article-title":"DJ-1 acts in parallel to the PINK1\/parkin pathway to control mitochondrial function and autophagy","volume":"20","author":"Thomas","year":"2011","journal-title":"Hum. Mol. Genet."},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1016\/S0002-9440(10)64553-1","article-title":"Alpha-synuclein promotes mitochondrial deficit and oxidative stress","volume":"157","author":"Hsu","year":"2000","journal-title":"Am. J. Pathol."},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"7006","DOI":"10.1523\/JNEUROSCI.22-16-07006.2002","article-title":"An in vitro model of Parkinson\u2019s disease: Linking mitochondrial impairment to altered alpha-synuclein metabolism and oxidative damage","volume":"22","author":"Sherer","year":"2002","journal-title":"J. Neurosci."},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"1931","DOI":"10.1093\/hmg\/dds003","article-title":"LRRK2 regulates mitochondrial dynamics and function through direct interaction with DLP1","volume":"21","author":"Wang","year":"2012","journal-title":"Hum. Mol. Genet."},{"key":"ref_117","doi-asserted-by":"crossref","first-page":"172","DOI":"10.1016\/j.nbd.2015.02.031","article-title":"Progressive dopaminergic alterations and mitochondrial abnormalities in LRRK2 G2019S knock-in mice","volume":"78","author":"Yue","year":"2015","journal-title":"Neurobiol. Dis."},{"key":"ref_118","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1089\/ars.2016.6634","article-title":"The essential role of Drp1 and its regulation by S-nitrosylation of Parkin in dopaminergic neurodegeneration: Implications for Parkinson\u2019s disease","volume":"25","author":"Zhang","year":"2016","journal-title":"Antioxid. Redox Signal."},{"key":"ref_119","doi-asserted-by":"crossref","first-page":"893","DOI":"10.1016\/j.cell.2011.10.018","article-title":"PINK1 and Parkin target Miro for phosphorylation and degradation to arrest mitochondrial motility","volume":"147","author":"Wang","year":"2011","journal-title":"Cell"},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"830","DOI":"10.1111\/j.1471-4159.2012.07734.x","article-title":"Parkinson\u2019s disease-associated DJ-1 mutations impair mitochondrial dynamics and cause mitochondrial dysfunction","volume":"121","author":"Wang","year":"2012","journal-title":"J. Neurochem."},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"795","DOI":"10.1083\/jcb.200809125","article-title":"Parkin is recruited selectively to impaired mitochondria and promotes their autophagy","volume":"183","author":"Narendra","year":"2008","journal-title":"J. Cell Biol."},{"key":"ref_122","doi-asserted-by":"crossref","first-page":"1162","DOI":"10.1038\/nature04779","article-title":"Drosophila PINK1 is required for mitochondrial function and interacts genetically with Parkin","volume":"441","author":"Clark","year":"2006","journal-title":"Nature"},{"key":"ref_123","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1523\/JNEUROSCI.2507-13.2014","article-title":"Alpha-synuclein is localized to mitochondria-associated ER membranes","volume":"34","author":"Rub","year":"2014","journal-title":"J. Neurosci."},{"key":"ref_124","doi-asserted-by":"crossref","first-page":"11649","DOI":"10.1074\/jbc.M110.144238","article-title":"Parkin ubiquitinates Drp1 for proteasome-dependent degradation: Implication of dysregulated mitochondrial dynamics in Parkinson disease","volume":"286","author":"Wang","year":"2011","journal-title":"J. Biol. Chem."},{"key":"ref_125","doi-asserted-by":"crossref","first-page":"1367","DOI":"10.1083\/jcb.201007013","article-title":"Proteasome and p97 mediate mitophagy and degradation of mitofusins induced by Parkin","volume":"191","author":"Tanaka","year":"2010","journal-title":"J. Cell Biol."},{"key":"ref_126","doi-asserted-by":"crossref","first-page":"243","DOI":"10.4161\/auto.7.2.14332","article-title":"PINK1-Parkin-dependent mitophagy involves ubiquitination of mitofusins 1 and 2 implications for Parkinson disease pathogenesis","volume":"7","author":"Gegg","year":"2011","journal-title":"Autophagy"},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"1697","DOI":"10.1093\/hmg\/ddt046","article-title":"Mitofusin 1 and mitofusin 2 are ubiquitinated in a PINK1\/Parkin-dependent manner upon induction of mitophagy (vol 19, pg 4861, 2010)","volume":"22","author":"Gegg","year":"2013","journal-title":"Hum. Mol. Genet."},{"key":"ref_128","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1002\/ana.24410","article-title":"Syndromic parkinsonism and dementia associated with OPA1 missense mutations","volume":"78","author":"Carelli","year":"2015","journal-title":"Ann. Neurol."},{"key":"ref_129","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1016\/S0079-6123(10)83006-7","article-title":"Control of mitochondrial integrity in Parkinson\u2019s disease","volume":"183","author":"Tocilescu","year":"2010","journal-title":"Prog. Brain Res."},{"key":"ref_130","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1126\/science.1134108","article-title":"Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis","volume":"314","author":"Neumann","year":"2006","journal-title":"Science"},{"key":"ref_131","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1097\/00005072-198409000-00001","article-title":"Fine structural observations of neurofilamentous changes in amyotrophic lateral sclerosis","volume":"43","author":"Hirano","year":"1984","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"ref_132","doi-asserted-by":"crossref","first-page":"869","DOI":"10.1038\/nm.4130","article-title":"The inhibition of TDP-43 mitochondrial localization blocks its neuronal toxicity","volume":"22","author":"Wang","year":"2016","journal-title":"Nat. Med."},{"key":"ref_133","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1590\/S0004-282X2012000100010","article-title":"Morphological abnormalities in mitochondria of the skin of patients with sporadic amyotrophic lateral sclerosis","volume":"70","author":"Rodriguez","year":"2012","journal-title":"Arq. Neuropsiquiatr."},{"key":"ref_134","doi-asserted-by":"crossref","unstructured":"Vande Velde, C., McDonald, K.K., Boukhedimi, Y., McAlonis-Downes, M., Lobsiger, C.S., Bel Hadj, S., Zandona, A., Julien, J.P., Shah, S.B., and Cleveland, D.W. (2011). Misfolded sod1 associated with motor neuron mitochondria alters mitochondrial shape and distribution prior to clinical onset. PLoS ONE, 6.","DOI":"10.1371\/journal.pone.0022031"},{"key":"ref_135","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1016\/j.nbd.2012.07.004","article-title":"Mutant SOD1G93A triggers mitochondrial fragmentation in spinal cord motor neurons: Neuroprotection by SIRT3 and PGC-1alpha","volume":"51","author":"Song","year":"2013","journal-title":"Neurobiol. Dis."},{"key":"ref_136","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1002\/brb3.142","article-title":"Characterization of early pathogenesis in the SOD1(G93A) mouse model of ALS: Part II, results and discussion","volume":"3","author":"Vinsant","year":"2013","journal-title":"Brain Behav."},{"key":"ref_137","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1002\/brb3.143","article-title":"Characterization of early pathogenesis in the SOD1(G93A) mouse model of ALS: Part I, background and methods","volume":"3","author":"Vinsant","year":"2013","journal-title":"Brain Behav."},{"key":"ref_138","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":"Magrane","year":"2014","journal-title":"Hum. Mol. Genet."},{"key":"ref_139","doi-asserted-by":"crossref","first-page":"222","DOI":"10.2174\/15672026113109990060","article-title":"Mitochondrial fusion and fission proteins expression dynamically change in a murine model of amyotrophic lateral sclerosis","volume":"10","author":"Liu","year":"2013","journal-title":"Curr. Neurovasc. Res."},{"key":"ref_140","doi-asserted-by":"crossref","first-page":"10851","DOI":"10.1523\/JNEUROSCI.1630-10.2010","article-title":"Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice","volume":"30","author":"Xu","year":"2010","journal-title":"J. Neurosci."},{"key":"ref_141","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1186\/1750-1326-6-73","article-title":"Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice","volume":"6","author":"Xu","year":"2011","journal-title":"Mol. Neurodegener."},{"key":"ref_142","doi-asserted-by":"crossref","first-page":"4706","DOI":"10.1093\/hmg\/ddt319","article-title":"The ALS disease-associated mutant TDP-43 impairs mitochondrial dynamics and function in motor neurons","volume":"22","author":"Wang","year":"2013","journal-title":"Hum. Mol. Genet."},{"key":"ref_143","doi-asserted-by":"crossref","unstructured":"Deng, J., Yang, M., Chen, Y., Chen, X., Liu, J., Sun, S., Cheng, H., Li, Y., Bigio, E.H., and Mesulam, M. (2015). FUS interacts with HSP60 to promote mitochondrial damage. PLoS Genet., 11.","DOI":"10.1371\/journal.pgen.1005357"},{"key":"ref_144","doi-asserted-by":"crossref","first-page":"2063","DOI":"10.1002\/stem.2388","article-title":"C9orf72 hexanucleotide expansions are associated with altered endoplasmic reticulum calcium homeostasis and stress granule formation in induced pluripotent stem cell-derived neurons from patients with amyotrophic lateral sclerosis and frontotemporal dementia","volume":"34","author":"Dafinca","year":"2016","journal-title":"Stem Cells"},{"key":"ref_145","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1016\/j.bbrc.2016.10.134","article-title":"Imbalance of mitochondrial dynamics in drosophila models of amyotrophic lateral sclerosis","volume":"481","author":"Altanbyek","year":"2016","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"ref_146","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":"ref_147","doi-asserted-by":"crossref","first-page":"1535","DOI":"10.1089\/ars.2009.2614","article-title":"Axonal mitochondrial clusters containing mutant SOD1 in transgenic models of ALS","volume":"11","author":"Lepanto","year":"2009","journal-title":"Antioxid. Redox Signal."},{"key":"ref_148","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":"Chapman","year":"2007","journal-title":"Hum. Mol. Genet."},{"key":"ref_149","doi-asserted-by":"crossref","first-page":"726","DOI":"10.1172\/JCI44867","article-title":"Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice","volume":"121","author":"Igaz","year":"2011","journal-title":"J. Clin. Investig."},{"key":"ref_150","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1007\/s12035-013-8427-5","article-title":"Overexpression of ALS-associated p.M337V human TDP-43 in mice worsens disease features compared to wild-type human TDP-43 mice","volume":"48","author":"Janssens","year":"2013","journal-title":"Mol. Neurobiol."},{"key":"ref_151","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1097\/00001756-200504250-00002","article-title":"Mutant superoxide dismutase disrupts cytoplasmic dynein in motor neurons","volume":"16","author":"Ligon","year":"2005","journal-title":"Neuroreport"},{"key":"ref_152","doi-asserted-by":"crossref","first-page":"1766","DOI":"10.1093\/hmg\/ddq058","article-title":"Stathmin, a microtubule-destabilizing protein, is dysregulated in spinal muscular atrophy","volume":"19","author":"Wen","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"ref_153","doi-asserted-by":"crossref","first-page":"100","DOI":"10.3389\/fnagi.2015.00100","article-title":"Miro1 deficiency in amyotrophic lateral sclerosis","volume":"7","author":"Zhang","year":"2015","journal-title":"Front. Aging Neurosci."},{"key":"ref_154","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1016\/j.neuron.2004.06.016","article-title":"Toxicity of familial ALS-linked SOD1 mutants from selective recruitment to spinal mitochondria","volume":"43","author":"Liu","year":"2004","journal-title":"Neuron"},{"key":"ref_155","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":"ref_156","doi-asserted-by":"crossref","first-page":"2463","DOI":"10.1523\/JNEUROSCI.4385-04.2005","article-title":"Mutant superoxide dismutase 1 forms aggregates in the brain mitochondrial matrix of amyotrophic lateral sclerosis mice","volume":"25","author":"Vijayvergiya","year":"2005","journal-title":"J. Neurosci."},{"key":"ref_157","doi-asserted-by":"crossref","first-page":"38388","DOI":"10.1074\/jbc.M105395200","article-title":"Subcellular distribution of superoxide dismutases (SOD) in rat liver: Cu,zn-SOD in mitochondria","volume":"276","author":"Fridovich","year":"2001","journal-title":"J. Biol. Chem."},{"key":"ref_158","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1016\/S0168-9525(03)00074-X","article-title":"Transcriptional abnormalities in Huntington disease","volume":"19","author":"Sugars","year":"2003","journal-title":"Trends Genet."},{"key":"ref_159","doi-asserted-by":"crossref","first-page":"143","DOI":"10.3233\/JHD-2012-120018","article-title":"New perspectives on the neuropathology in Huntington\u2019s disease in the human brain and its relation to symptom variation","volume":"1","author":"Waldvogel","year":"2012","journal-title":"J. Huntingt. Dis."},{"key":"ref_160","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1093\/hmg\/ddr475","article-title":"Mutant Huntingtin\u2019s interaction with mitochondrial protein Drp1 impairs mitochondrial biogenesis and causes defective axonal transport and synaptic degeneration in Huntington\u2019s disease","volume":"21","author":"Shirendeb","year":"2012","journal-title":"Hum. Mol. Genet."},{"key":"ref_161","doi-asserted-by":"crossref","first-page":"3919","DOI":"10.1093\/hmg\/ddq306","article-title":"Mitochondrial loss, dysfunction and altered dynamics in Huntington\u2019s disease","volume":"19","author":"Kim","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"ref_162","doi-asserted-by":"crossref","first-page":"2484","DOI":"10.1016\/j.bbadis.2015.08.012","article-title":"HDAC6 inhibition induces mitochondrial fusion, autophagic flux and reduces diffuse mutant huntingtin in striatal neurons","volume":"1852","author":"Soares","year":"2015","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_163","doi-asserted-by":"crossref","first-page":"1173","DOI":"10.1089\/ars.2012.4928","article-title":"S-nitrosylation of dynamin-related protein 1 mediates mutant huntingtin-induced mitochondrial fragmentation and neuronal injury in Huntington\u2019s disease","volume":"19","author":"Haun","year":"2013","journal-title":"Antioxid. Redox Signal."},{"key":"ref_164","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1111\/j.1476-5381.2012.01994.x","article-title":"3-Nitropropionic acid induces autophagy by forming mitochondrial permeability transition pores rather than activatiing the mitochondrial fission pathway","volume":"168","author":"Solesio","year":"2013","journal-title":"Br. J. Pharmacol."},{"key":"ref_165","doi-asserted-by":"crossref","first-page":"899","DOI":"10.1038\/cdd.2009.22","article-title":"Complex II inhibition by 3-NP causes mitochondrial fragmentation and neuronal cell death via an NMDA- and ROS-dependent pathway","volume":"16","author":"Liot","year":"2009","journal-title":"Cell Death Differ."},{"key":"ref_166","doi-asserted-by":"crossref","first-page":"4181","DOI":"10.1523\/JNEUROSCI.13-10-04181.1993","article-title":"Neurochemical and histologic characterization of striatal excitotoxic lesions produced by the mitochondrial toxin 3-nitropropionic acid","volume":"13","author":"Beal","year":"1993","journal-title":"J. Neurosci."},{"key":"ref_167","doi-asserted-by":"crossref","first-page":"102","DOI":"10.1126\/science.1171091","article-title":"S-nitrosylation of Drp1 mediates beta-amyloid-related mitochondrial fission and neuronal injury","volume":"324","author":"Cho","year":"2009","journal-title":"Science"},{"key":"ref_168","doi-asserted-by":"crossref","first-page":"8195","DOI":"10.1128\/MCB.24.18.8195-8209.2004","article-title":"Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro","volume":"24","author":"Trushina","year":"2004","journal-title":"Mol. Cell. Biol."},{"key":"ref_169","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1016\/j.cell.2004.06.018","article-title":"Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules","volume":"118","author":"Gauthier","year":"2004","journal-title":"Cell"},{"key":"ref_170","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1016\/j.bbadis.2009.09.003","article-title":"Mitochondria, oxidative metabolism and cell death in stroke","volume":"1802","author":"Sims","year":"2010","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_171","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1097\/01.WCB.0000111614.19196.04","article-title":"Pathophysiology of cerebral ischemia and brain trauma: Similarities and differences","volume":"24","author":"Bramlett","year":"2004","journal-title":"J. Cereb. Blood Flow Metab."},{"key":"ref_172","doi-asserted-by":"crossref","first-page":"5244","DOI":"10.1038\/ncomms6244","article-title":"Drp1 inhibition attenuates neurotoxicity and dopamine release deficits in vivo","volume":"5","author":"Rappold","year":"2014","journal-title":"Nat. Commun."},{"key":"ref_173","doi-asserted-by":"crossref","first-page":"5371","DOI":"10.1172\/JCI70911","article-title":"Inhibition of mitochondrial fragmentation diminishes Huntington\u2019s disease-associated neurodegeneration","volume":"123","author":"Guo","year":"2013","journal-title":"J. Clin. Investig."},{"key":"ref_174","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1007\/s12035-009-8095-7","article-title":"Abnormal mitochondrial dynamics\u2014A novel therapeutic target for Alzheimer\u2019s disease?","volume":"41","author":"Su","year":"2010","journal-title":"Mol. Neurobiol."}],"container-title":["Antioxidants"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2076-3921\/6\/2\/25\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T18:32:02Z","timestamp":1760207522000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2076-3921\/6\/2\/25"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,4,5]]},"references-count":174,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2017,6]]}},"alternative-id":["antiox6020025"],"URL":"https:\/\/doi.org\/10.3390\/antiox6020025","relation":{},"ISSN":["2076-3921"],"issn-type":[{"value":"2076-3921","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,4,5]]}}}