{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T14:15:26Z","timestamp":1792764926673,"version":"4.5.0"},"reference-count":148,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2024,12,30]],"date-time":"2024-12-30T00:00:00Z","timestamp":1735516800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Israel Science Foundation","award":["ISF #284\/19"],"award-info":[{"award-number":["ISF #284\/19"]}]},{"name":"Israel Science Foundation","award":["#485\/24"],"award-info":[{"award-number":["#485\/24"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Biomolecules"],"abstract":"<jats:p>Voltage-Dependent Anion Channel 1 (VDAC1) is a mitochondrial outer membrane protein that plays a crucial role in regulating cellular energy metabolism and apoptosis by mediating the exchange of ions and metabolites between mitochondria and the cytosol. Mitochondrial dysfunction and oxidative stress are central features of neurodegenerative diseases. The pivotal functions of VDAC1 in controlling mitochondrial membrane permeability, regulating calcium balance, and facilitating programmed cell death pathways, position it as a key determinant in the delicate balance between neuronal viability and degeneration. Accordingly, increasing evidence suggests that VDAC1 is implicated in the pathophysiology of neurodegenerative diseases, including Alzheimer\u2019s disease (AD), Parkinson\u2019s disease (PD), amyotrophic lateral sclerosis (ALS), and others. This review summarizes the current findings on the contribution of VDAC1 to neurodegeneration, focusing on its interactions with disease-specific proteins, such as amyloid-\u03b2, \u03b1-synuclein, and mutant SOD1. By unraveling the complex involvement of VDAC1 in neurodegenerative processes, this review highlights potential avenues for future research and drug development aimed at alleviating mitochondrial-related neurodegeneration.<\/jats:p>","DOI":"10.3390\/biom15010033","type":"journal-article","created":{"date-parts":[[2024,12,31]],"date-time":"2024-12-31T12:37:19Z","timestamp":1735648639000},"page":"33","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":33,"title":["VDAC1: A Key Player in the Mitochondrial Landscape of Neurodegeneration"],"prefix":"10.3390","volume":"15","author":[{"given":"Shirel","family":"Argueti-Ostrovsky","sequence":"first","affiliation":[{"name":"Department of Physiology and Cell Biology, Faculty of Health Sciences, Ben-Gurion University of the Negev, P.O. Box 653, Beer Sheva 84105, Israel"},{"name":"The School of Brain Sciences and Cognition, Ben-Gurion University of the Negev, P.O. Box 653, Beer Sheva 84105, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1795-6653","authenticated-orcid":false,"given":"Shir","family":"Barel","sequence":"additional","affiliation":[{"name":"Department of Physiology and Cell Biology, Faculty of Health Sciences, Ben-Gurion University of the Negev, P.O. Box 653, Beer Sheva 84105, Israel"},{"name":"The School of Brain Sciences and Cognition, Ben-Gurion University of the Negev, P.O. Box 653, Beer Sheva 84105, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joy","family":"Kahn","sequence":"additional","affiliation":[{"name":"Department of Physiology and Cell Biology, Faculty of Health Sciences, Ben-Gurion University of the Negev, P.O. Box 653, Beer Sheva 84105, Israel"},{"name":"The School of Brain Sciences and Cognition, Ben-Gurion University of the Negev, P.O. Box 653, Beer Sheva 84105, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8289-7653","authenticated-orcid":false,"given":"Adrian","family":"Israelson","sequence":"additional","affiliation":[{"name":"Department of Physiology and Cell Biology, Faculty of Health Sciences, Ben-Gurion University of the Negev, P.O. Box 653, Beer Sheva 84105, Israel"},{"name":"The School of Brain Sciences and Cognition, Ben-Gurion University of the Negev, P.O. Box 653, Beer Sheva 84105, Israel"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,12,30]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"745","DOI":"10.1038\/s41556-018-0124-1","article-title":"The multifaceted contributions of mitochondria to cellular metabolism","volume":"20","author":"Spinelli","year":"2018","journal-title":"Nat. Cell Biol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1038\/s41392-023-01547-9","article-title":"Mitochondrial dynamics in health and disease: Mechanisms and potential targets","volume":"8","author":"Chen","year":"2023","journal-title":"Signal Transduct. Target. Ther."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"346","DOI":"10.1016\/j.tibs.2024.01.011","article-title":"Mitochondria in disease: Changes in shapes and dynamics","volume":"49","author":"Jenkins","year":"2024","journal-title":"Trends Biochem. Sci."},{"key":"ref_4","doi-asserted-by":"crossref","unstructured":"Shoshan-Barmatz, V., Shteinfer-Kuzmine, A., and Verma, A. (2020). VDAC1 at the Intersection of Cell Metabolism, Apoptosis, and Diseases. Biomolecules, 10.","DOI":"10.3390\/biom10111485"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1007\/s002329900235","article-title":"Regulation of metabolite flux through voltage-gating of VDAC channels","volume":"157","author":"Hodge","year":"1997","journal-title":"J. Membr. Biol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1954","DOI":"10.1016\/S0006-3495(97)78841-6","article-title":"VDAC channels mediate and gate the flow of ATP: Implications for the regulation of mitochondrial function","volume":"72","author":"Rostovtseva","year":"1997","journal-title":"Biophys. J."},{"key":"ref_7","doi-asserted-by":"crossref","unstructured":"Magri, A., Reina, S., and De Pinto, V. (2018). VDAC1 as Pharmacological Target in Cancer and Neurodegeneration: Focus on Its Role in Apoptosis. Front. Chem., 6.","DOI":"10.3389\/fchem.2018.00108"},{"key":"ref_8","first-page":"1061","article-title":"Pore-Forming VDAC Proteins of the Outer Mitochondrial Membrane: Regulation and Pathophysiological Role","volume":"89","author":"Belosludtseva","year":"2024","journal-title":"Biochemistry"},{"key":"ref_9","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_10","doi-asserted-by":"crossref","unstructured":"Singh, A., Kukreti, R., Saso, L., and Kukreti, S. (2019). Oxidative Stress: A Key Modulator in Neurodegenerative Diseases. Molecules, 24.","DOI":"10.3390\/molecules24081583"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1038\/nrd1330","article-title":"Neurodegenerative diseases and oxidative stress","volume":"3","author":"Barnham","year":"2004","journal-title":"Nat. Rev. Drug Discov."},{"key":"ref_12","first-page":"2922","article-title":"The expanding role of mitochondria in apoptosis","volume":"15","author":"Wang","year":"2001","journal-title":"Genes Dev."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1016\/S0005-2728(98)00167-4","article-title":"Apoptosis in neurodegenerative diseases: The role of mitochondria","volume":"1410","author":"Tatton","year":"1999","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1016\/j.phrs.2018.03.010","article-title":"VDAC1, mitochondrial dysfunction, and Alzheimer\u2019s disease","volume":"131","author":"Gupta","year":"2018","journal-title":"Pharmacol. Res."},{"key":"ref_15","first-page":"4435","article-title":"VDAC1 as a Player in Mitochondria-Mediated Apoptosis and Target for Modulating Apoptosis","volume":"24","author":"Krelin","year":"2017","journal-title":"Curr. Med. Chem."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1016\/j.ceca.2006.06.005","article-title":"Localization of the voltage-dependent anion channel-1 Ca2+-binding sites","volume":"41","author":"Israelson","year":"2007","journal-title":"Cell Calcium"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"196","DOI":"10.1016\/j.ceca.2007.05.006","article-title":"Mapping the ruthenium red-binding site of the voltage-dependent anion channel-1","volume":"43","author":"Israelson","year":"2008","journal-title":"Cell Calcium"},{"key":"ref_18","doi-asserted-by":"crossref","unstructured":"Hu, H., Guo, L., Overholser, J., and Wang, X. (2022). Mitochondrial VDAC1: A Potential Therapeutic Target of Inflammation-Related Diseases and Clinical Opportunities. Cells, 11.","DOI":"10.3390\/cells11193174"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"15370","DOI":"10.1073\/pnas.0808115105","article-title":"Structure of the human voltage-dependent anion channel","volume":"105","author":"Bayrhuber","year":"2008","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"17742","DOI":"10.1073\/pnas.0809634105","article-title":"The crystal structure of mouse VDAC1 at 2.3 A resolution reveals mechanistic insights into metabolite gating","volume":"105","author":"Ujwal","year":"2008","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1042\/BJ20112079","article-title":"Structure-based analysis of VDAC1: N-terminus location, translocation, channel gating and association with anti-apoptotic proteins","volume":"444","author":"Geula","year":"2012","journal-title":"Biochem. J."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"11","DOI":"10.15698\/cst2017.10.104","article-title":"VDAC1 at the crossroads of cell metabolism, apoptosis and cell stress","volume":"1","author":"Maldonado","year":"2017","journal-title":"Cell Stress"},{"key":"ref_23","doi-asserted-by":"crossref","unstructured":"Lei, P., Ayton, S., and Bush, A.I. (2021). The essential elements of Alzheimer\u2019s disease. J. Biol. Chem., 296.","DOI":"10.1074\/jbc.REV120.008207"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1016\/j.bbadis.2012.09.003","article-title":"Is the mitochondrial outermembrane protein VDAC1 therapeutic target for Alzheimer\u2019s disease?","volume":"1832","author":"Reddy","year":"2013","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1577","DOI":"10.1016\/S0140-6736(20)32205-4","article-title":"Alzheimer\u2019s disease","volume":"397","author":"Scheltens","year":"2021","journal-title":"Lancet"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"311","DOI":"10.3233\/JAD-2010-1221","article-title":"Alzheimer\u2019s disease and the amyloid-beta peptide","volume":"19","author":"Murphy","year":"2010","journal-title":"J. Alzheimer\u2019s Dis."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1774","DOI":"10.1126\/science.1197623","article-title":"Decreased clearance of CNS beta-amyloid in Alzheimer\u2019s disease","volume":"330","author":"Mawuenyega","year":"2010","journal-title":"Science"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1186\/alzrt187","article-title":"Evidence for impaired amyloid beta clearance in Alzheimer\u2019s disease","volume":"5","author":"Wildsmith","year":"2013","journal-title":"Alzheimer\u2019s Res. Ther."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1016\/j.bbadis.2011.10.011","article-title":"Abnormal mitochondrial dynamics and synaptic degeneration as early events in Alzheimer\u2019s disease: Implications to mitochondria-targeted antioxidant therapeutics","volume":"1822","author":"Reddy","year":"2012","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"789","DOI":"10.1126\/science.1074069","article-title":"Alzheimer\u2019s disease is a synaptic failure","volume":"298","author":"Selkoe","year":"2002","journal-title":"Science"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"1630","DOI":"10.1016\/j.bbadis.2011.08.012","article-title":"Brain aging, Alzheimer\u2019s disease, and mitochondria","volume":"1812","author":"Swerdlow","year":"2011","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"S253","DOI":"10.3233\/JAD-2012-129005","article-title":"Abnormal mitochondrial dynamics in the pathogenesis of Alzheimer\u2019s disease","volume":"33","author":"Zhu","year":"2013","journal-title":"J. Alzheimer\u2019s Dis."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"619.e25","DOI":"10.1016\/j.neurobiolaging.2011.02.007","article-title":"Truncated tau and Abeta cooperatively impair mitochondria in primary neurons","volume":"33","author":"Quintanilla","year":"2012","journal-title":"Neurobiol. Aging"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"1556","DOI":"10.1016\/j.bbadis.2014.05.025","article-title":"Early alterations in energy metabolism in the hippocampus of APPswe\/PS1dE9 mouse model of Alzheimer\u2019s disease","volume":"1842","author":"Pedros","year":"2014","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1385\/NMM:5:2:147","article-title":"Differential expression of oxidative phosphorylation genes in patients with Alzheimer\u2019s disease: Implications for early mitochondrial dysfunction and oxidative damage","volume":"5","author":"Manczak","year":"2004","journal-title":"Neuromol. Med."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"1659","DOI":"10.1089\/ars.2007.1763","article-title":"Mitochondrial dysfunction: The first domino in brain aging and Alzheimer\u2019s disease?","volume":"9","author":"Leuner","year":"2007","journal-title":"Antioxid. Redox Signal."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"1574","DOI":"10.1016\/j.neurobiolaging.2007.12.005","article-title":"Mitochondrial dysfunction: An early event in Alzheimer pathology accumulates with age in AD transgenic mice","volume":"30","author":"Hauptmann","year":"2009","journal-title":"Neurobiol. Aging"},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"S569","DOI":"10.3233\/JAD-2010-100357","article-title":"Role of mitochondrial amyloid-beta in Alzheimer\u2019s disease","volume":"20","author":"Chen","year":"2010","journal-title":"J. Alzheimer\u2019s Dis."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1016\/j.expneurol.2009.03.042","article-title":"Amyloid beta, mitochondrial structural and functional dynamics in Alzheimer\u2019s disease","volume":"218","author":"Reddy","year":"2009","journal-title":"Exp. Neurol."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"1434","DOI":"10.1089\/ars.2011.4149","article-title":"Mitochondria and cell bioenergetics: Increasingly recognized components and a possible etiologic cause of Alzheimer\u2019s disease","volume":"16","author":"Swerdlow","year":"2012","journal-title":"Antioxid. Redox Signal."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"5131","DOI":"10.1093\/hmg\/dds360","article-title":"Abnormal interaction of VDAC1 with amyloid beta and phosphorylated tau causes mitochondrial dysfunction in Alzheimer\u2019s disease","volume":"21","author":"Manczak","year":"2012","journal-title":"Hum. Mol. Genet."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"1450","DOI":"10.1002\/jcb.23062","article-title":"Effect of beta-amyloid (25\u201335) on mitochondrial function and expression of mitochondrial permeability transition pore proteins in rat hippocampal neurons","volume":"112","author":"Ren","year":"2011","journal-title":"J. Cell. Biochem."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"195","DOI":"10.3233\/JAD-2010-100966","article-title":"Enhanced expression of the voltage-dependent anion channel 1 (VDAC1) in Alzheimer\u2019s disease transgenic mice: An insight into the pathogenic effects of amyloid-beta","volume":"23","author":"Vilarino","year":"2011","journal-title":"J. Alzheimer\u2019s Dis."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1016\/j.nbd.2005.10.004","article-title":"Identification of nitrated proteins in Alzheimer\u2019s disease brain using a redox proteomics approach","volume":"22","author":"Sultana","year":"2006","journal-title":"Neurobiol. Dis."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"30670","DOI":"10.1074\/jbc.M115.691493","article-title":"The Voltage-dependent Anion Channel 1 Mediates Amyloid beta Toxicity and Represents a Potential Target for Alzheimer Disease Therapy","volume":"290","author":"Smilansky","year":"2015","journal-title":"J. Biol. Chem."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"274","DOI":"10.1007\/s10354-011-0887-5","article-title":"Apoptogenic interactions of plasmalemmal type-1 VDAC and Abeta peptides via GxxxG motifs induce Alzheimer\u2019s disease\u2014A basic model of apoptosis?","volume":"161","author":"Thinnes","year":"2011","journal-title":"Wien. Med. Wochenschr."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1007\/s11064-006-9128-5","article-title":"Glycogen synthase kinase-3 (GSK3): Inflammation, diseases, and therapeutics","volume":"32","author":"Jope","year":"2007","journal-title":"Neurochem. Res."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"10545","DOI":"10.1158\/0008-5472.CAN-05-1925","article-title":"Activation of glycogen synthase kinase 3beta disrupts the binding of hexokinase II to mitochondria by phosphorylating voltage-dependent anion channel and potentiates chemotherapy-induced cytotoxicity","volume":"65","author":"Pastorino","year":"2005","journal-title":"Cancer Res."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"1906","DOI":"10.1242\/jcs.040188","article-title":"The VDAC1 N-terminus is essential both for apoptosis and the protective effect of anti-apoptotic proteins","volume":"122","author":"Arbel","year":"2009","journal-title":"J. Cell Sci."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"3946","DOI":"10.1074\/jbc.M803614200","article-title":"Voltage-dependent anion channel 1-based peptides interact with hexokinase to prevent its anti-apoptotic activity","volume":"284","author":"Arzoine","year":"2009","journal-title":"J. Biol. Chem."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"24986","DOI":"10.1074\/jbc.M116.744284","article-title":"Novel Compounds Targeting the Mitochondrial Protein VDAC1 Inhibit Apoptosis and Protect against Mitochondrial Dysfunction","volume":"291","author":"Shalev","year":"2016","journal-title":"J. Biol. Chem."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1016\/j.cmet.2018.09.008","article-title":"Preserving Insulin Secretion in Diabetes by Inhibiting VDAC1 Overexpression and Surface Translocation in beta Cells","volume":"29","author":"Zhang","year":"2019","journal-title":"Cell Metab."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"1531","DOI":"10.1126\/science.aav4011","article-title":"VDAC oligomers form mitochondrial pores to release mtDNA fragments and promote lupus-like disease","volume":"366","author":"Kim","year":"2019","journal-title":"Science"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"726","DOI":"10.1016\/j.ymthe.2021.06.024","article-title":"The role of the mitochondrial protein VDAC1 in inflammatory bowel disease: A potential therapeutic target","volume":"30","author":"Verma","year":"2022","journal-title":"Mol. Ther."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1186\/s40035-022-00329-7","article-title":"Targeting the overexpressed mitochondrial protein VDAC1 in a mouse model of Alzheimer\u2019s disease protects against mitochondrial dysfunction and mitigates brain pathology","volume":"11","author":"Verma","year":"2022","journal-title":"Transl. Neurodegener."},{"key":"ref_56","doi-asserted-by":"crossref","unstructured":"Shteinfer-Kuzmine, A., Argueti-Ostrovsky, S., Leyton-Jaimes, M.F., Anand, U., Abu-Hamad, S., Zalk, R., Shoshan-Barmatz, V., and Israelson, A. (2022). Targeting the Mitochondrial Protein VDAC1 as a Potential Therapeutic Strategy in ALS. Int. J. Mol. Sci., 23.","DOI":"10.3390\/ijms23179946"},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"636","DOI":"10.1016\/j.cell.2020.09.020","article-title":"TDP-43 Triggers Mitochondrial DNA Release via mPTP to Activate cGAS\/STING in ALS","volume":"183","author":"Yu","year":"2020","journal-title":"Cell"},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"3611","DOI":"10.1007\/s00018-019-03386-w","article-title":"Molecular mechanism of olesoxime-mediated neuroprotection through targeting alpha-synuclein interaction with mitochondrial VDAC","volume":"77","author":"Rovini","year":"2020","journal-title":"Cell. Mol. Life Sci."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"709","DOI":"10.1124\/jpet.107.123000","article-title":"Identification and characterization of cholest-4-en-3-one, oxime (TRO19622), a novel drug candidate for amyotrophic lateral sclerosis","volume":"322","author":"Bordet","year":"2007","journal-title":"J. Pharmacol. Exp. Ther."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1016\/j.biocel.2015.01.015","article-title":"The therapeutic effects of 4-phenylbutyric acid in maintaining proteostasis","volume":"61","author":"Kolb","year":"2015","journal-title":"Int. J. Biochem. Cell Biol."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"3385","DOI":"10.1007\/s11064-022-03691-0","article-title":"4-Phenylbutyrate Mitigates the Motor Impairment and Dopaminergic Neuronal Death During Parkinson\u2019s Disease Pathology via Targeting VDAC1 Mediated Mitochondrial Function and Astrocytes Activation","volume":"47","author":"Tiwari","year":"2022","journal-title":"Neurochem. Res."},{"key":"ref_62","doi-asserted-by":"crossref","unstructured":"Shteinfer-Kuzmine, A., Argueti, S., Gupta, R., Shvil, N., Abu-Hamad, S., Gropper, Y., Hoeber, J., Magri, A., Messina, A., and Kozlova, E.N. (2019). A VDAC1-Derived N-Terminal Peptide Inhibits Mutant SOD1-VDAC1 Interactions and Toxicity in the SOD1 Model of ALS. Front. Cell. Neurosci., 13.","DOI":"10.3389\/fncel.2019.00346"},{"key":"ref_63","doi-asserted-by":"crossref","unstructured":"Magri, A., Belfiore, R., Reina, S., Tomasello, M.F., Di Rosa, M.C., Guarino, F., Leggio, L., De Pinto, V., and Messina, A. (2016). Hexokinase I N-terminal based peptide prevents the VDAC1-SOD1 G93A interaction and re-establishes ALS cell viability. Sci. Rep., 6.","DOI":"10.1038\/srep34802"},{"key":"ref_64","doi-asserted-by":"crossref","unstructured":"Magri, A., Risiglione, P., Caccamo, A., Formicola, B., Tomasello, M.F., Arrigoni, C., Zimbone, S., Guarino, F., Re, F., and Messina, A. (2021). Small Hexokinase 1 Peptide against Toxic SOD1 G93A Mitochondrial Accumulation in ALS Rescues the ATP-Related Respiration. Biomedicines, 9.","DOI":"10.3390\/biomedicines9080948"},{"key":"ref_65","doi-asserted-by":"crossref","unstructured":"Vijayan, M., and Reddy, P.H. (2022). Reduced VDAC1, Maintained Mitochondrial Dynamics and Enhanced Mitochondrial Biogenesis in a Transgenic Tau Mouse Model of Alzheimer\u2019s Disease. Int. J. Mol. Sci., 23.","DOI":"10.3390\/ijms23158561"},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1016\/S0140-6736(23)01419-8","article-title":"The epidemiology of Parkinson\u2019s disease","volume":"403","author":"Darweesh","year":"2024","journal-title":"Lancet"},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"45","DOI":"10.3233\/JPD-212922","article-title":"Preventing Parkinson\u2019s Disease: An Environmental Agenda","volume":"12","author":"Goldman","year":"2022","journal-title":"J. Parkinson\u2019s Dis."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"2284","DOI":"10.1016\/S0140-6736(21)00218-X","article-title":"Parkinson\u2019s disease","volume":"397","author":"Bloem","year":"2021","journal-title":"Lancet"},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"65","DOI":"10.2741\/S415","article-title":"Parkinson\u2019s disease: A review","volume":"6","author":"Beitz","year":"2014","journal-title":"Front. Biosci. (Schol. Ed.)"},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"R183","DOI":"10.1093\/hmg\/ddm159","article-title":"Parkinson\u2019s disease","volume":"16","author":"Thomas","year":"2007","journal-title":"Hum. Mol. Genet."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"a008888","DOI":"10.1101\/cshperspect.a008888","article-title":"Genetics of Parkinson\u2019s disease","volume":"2","author":"Klein","year":"2012","journal-title":"Cold Spring Harb. Perspect. Med."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1016\/j.ymgme.2003.11.004","article-title":"Glucocerebrosidase mutations in subjects with parkinsonism","volume":"81","author":"Lwin","year":"2004","journal-title":"Mol. Genet. Metab."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1007\/s00441-018-2817-y","article-title":"Genetic risk factors in Parkinson\u2019s disease","volume":"373","author":"Billingsley","year":"2018","journal-title":"Cell Tissue Res."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1016\/S0197-4580(02)00065-9","article-title":"Staging of brain pathology related to sporadic Parkinson\u2019s disease","volume":"24","author":"Braak","year":"2003","journal-title":"Neurobiol. Aging"},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"839","DOI":"10.1038\/42166","article-title":"Alpha-synuclein in Lewy bodies","volume":"388","author":"Spillantini","year":"1997","journal-title":"Nature"},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"791","DOI":"10.1038\/s41586-022-05319-3","article-title":"Structures of alpha-synuclein filaments from human brains with Lewy pathology","volume":"610","author":"Yang","year":"2022","journal-title":"Nature"},{"key":"ref_77","doi-asserted-by":"crossref","unstructured":"Guo, Y., Sun, Y., Song, Z., Zheng, W., Xiong, W., Yang, Y., Yuan, L., and Deng, H. (2021). Genetic Analysis and Literature Review of SNCA Variants in Parkinson\u2019s Disease. Front. Aging Neurosci., 13.","DOI":"10.3389\/fnagi.2021.648151"},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1016\/S0140-6736(23)01478-2","article-title":"The pathogenesis of Parkinson\u2019s disease","volume":"403","author":"Morris","year":"2024","journal-title":"Lancet"},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"4459","DOI":"10.1007\/s10072-021-05551-1","article-title":"A systematic review of molecular approaches that link mitochondrial dysfunction and neuroinflammation in Parkinson\u2019s disease","volume":"42","author":"Mani","year":"2021","journal-title":"Neurol. Sci."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"S11","DOI":"10.3233\/JPD-179006","article-title":"The MPTP Story","volume":"7","author":"Langston","year":"2017","journal-title":"J. Parkinson\u2019s Dis."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"1301","DOI":"10.1038\/81834","article-title":"Chronic systemic pesticide exposure reproduces features of Parkinson\u2019s disease","volume":"3","author":"Betarbet","year":"2000","journal-title":"Nat. Neurosci."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"4665530","DOI":"10.1155\/2022\/4665530","article-title":"The Potential Role of Voltage-Dependent Anion Channel in the Treatment of Parkinson\u2019s Disease","volume":"2022","author":"He","year":"2022","journal-title":"Oxid. Med. Cell. Longev."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"4281","DOI":"10.1073\/pnas.1909814117","article-title":"Decision between mitophagy and apoptosis by Parkin via VDAC1 ubiquitination","volume":"117","author":"Ham","year":"2020","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1016\/j.neuron.2014.12.007","article-title":"The roles of PINK1, parkin, and mitochondrial fidelity in Parkinson\u2019s disease","volume":"85","author":"Pickrell","year":"2015","journal-title":"Neuron"},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"717","DOI":"10.1136\/thoraxjnl-2019-214158","article-title":"Parkin, an E3 ubiquitin ligase, enhances airway mitochondrial DNA release and inflammation","volume":"75","author":"Dimasuay","year":"2020","journal-title":"Thorax"},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"1363","DOI":"10.1038\/cr.2009.98","article-title":"Outer membrane VDAC1 controls permeability transition of the inner mitochondrial membrane in cellulo during stress-induced apoptosis","volume":"19","author":"Tomasello","year":"2009","journal-title":"Cell Res."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"25322","DOI":"10.1073\/pnas.1906565116","article-title":"DJ-1 regulates the integrity and function of ER-mitochondria association through interaction with IP3R3-Grp75-VDAC1","volume":"116","author":"Liu","year":"2019","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"eaan5835","DOI":"10.1126\/science.aan5835","article-title":"Here, there, and everywhere: The importance of ER membrane contact sites","volume":"361","author":"Wu","year":"2018","journal-title":"Science"},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"e201912501","DOI":"10.1085\/jgp.201912501","article-title":"A lower affinity to cytosolic proteins reveals VDAC3 isoform-specific role in mitochondrial biology","volume":"152","author":"Bergdoll","year":"2020","journal-title":"J. Gen. Physiol."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"18467","DOI":"10.1074\/jbc.M115.641746","article-title":"alpha-Synuclein Shows High Affinity Interaction with Voltage-dependent Anion Channel, Suggesting Mechanisms of Mitochondrial Regulation and Toxicity in Parkinson Disease","volume":"290","author":"Rostovtseva","year":"2015","journal-title":"J. Biol. Chem."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1093\/abbs\/gms114","article-title":"Voltage-dependent anion channel involved in the alpha-synuclein-induced dopaminergic neuron toxicity in rats","volume":"45","author":"Lu","year":"2013","journal-title":"Acta Biochim. Biophys. Sin."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"102355","DOI":"10.1016\/j.ceca.2021.102355","article-title":"alpha-Synuclein emerges as a potent regulator of VDAC-facilitated calcium transport","volume":"95","author":"Rosencrans","year":"2021","journal-title":"Cell Calcium"},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.nbd.2014.05.003","article-title":"Abnormal alpha-synuclein reduces nigral voltage-dependent anion channel 1 in sporadic and experimental Parkinson\u2019s disease","volume":"69","author":"Chu","year":"2014","journal-title":"Neurobiol. Dis."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"1816","DOI":"10.1016\/j.bbadis.2014.06.033","article-title":"Altered dopamine homeostasis differentially affects mitochondrial voltage-dependent anion channels turnover","volume":"1842","author":"Alberio","year":"2014","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"746","DOI":"10.1007\/s11064-008-9844-0","article-title":"Protective effects of asiatic acid on rotenone- or H2O2-induced injury in SH-SY5Y cells","volume":"34","author":"Xiong","year":"2009","journal-title":"Neurochem. Res."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"1259","DOI":"10.1111\/j.1471-4159.2005.03442.x","article-title":"Proteomic analysis of parkin knockout mice: Alterations in energy metabolism, protein handling and synaptic function","volume":"95","author":"Periquet","year":"2005","journal-title":"J. Neurochem."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"1026","DOI":"10.1007\/s12031-021-01962-z","article-title":"6-Hydroxydopamine Induces Neurodegeneration in Terminally Differentiated SH-SY5Y Neuroblastoma Cells via Enrichment of the Nucleosomal Degradation Pathway: A Global Proteomics Approach","volume":"72","author":"Magalingam","year":"2022","journal-title":"J. Mol. Neurosci."},{"key":"ref_98","doi-asserted-by":"crossref","unstructured":"Feng, S., Gui, J., Qin, B., Ye, J., Zhao, Q., Guo, A., Sang, M., and Sun, X. (2024). Resveratrol Inhibits VDAC1-Mediated Mitochondrial Dysfunction to Mitigate Pathological Progression in Parkinson\u2019s Disease Model. Mol. Neurobiol.","DOI":"10.1007\/s12035-024-04234-0"},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1038\/s41573-022-00612-2","article-title":"Amyotrophic lateral sclerosis: A neurodegenerative disorder poised for successful therapeutic translation","volume":"22","author":"Mead","year":"2023","journal-title":"Nat. Rev. Drug Discov."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1016\/j.neuron.2015.02.034","article-title":"Macrophage migration inhibitory factor as a chaperone inhibiting accumulation of misfolded SOD1","volume":"86","author":"Israelson","year":"2015","journal-title":"Neuron"},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"1363","DOI":"10.1016\/S0140-6736(22)01272-7","article-title":"Amyotrophic lateral sclerosis","volume":"400","author":"Feldman","year":"2022","journal-title":"Lancet"},{"key":"ref_102","doi-asserted-by":"crossref","unstructured":"Wang, H., Guan, L., and Deng, M. (2023). Recent progress of the genetics of amyotrophic lateral sclerosis and challenges of gene therapy. Front. Neurosci., 17.","DOI":"10.3389\/fnins.2023.1170996"},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"416","DOI":"10.1016\/j.neuron.2013.07.033","article-title":"Converging mechanisms in ALS and FTD: Disrupted RNA and protein homeostasis","volume":"79","author":"Ling","year":"2013","journal-title":"Neuron"},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"1918","DOI":"10.1111\/ene.14393","article-title":"Amyotrophic lateral sclerosis: A clinical review","volume":"27","author":"Masrori","year":"2020","journal-title":"Eur. J. Neurol."},{"key":"ref_105","doi-asserted-by":"crossref","unstructured":"Ramesh, N., and Pandey, U.B. (2017). Autophagy Dysregulation in ALS: When Protein Aggregates Get Out of Hand. Front. Mol. Neurosci., 10.","DOI":"10.3389\/fnmol.2017.00263"},{"key":"ref_106","doi-asserted-by":"crossref","unstructured":"Carri, M.T., Valle, C., Bozzo, F., and Cozzolino, M. (2015). Oxidative stress and mitochondrial damage: Importance in non-SOD1 ALS. Front. Cell. Neurosci., 9.","DOI":"10.3389\/fncel.2015.00041"},{"key":"ref_107","doi-asserted-by":"crossref","unstructured":"Le Gall, L., Anakor, E., Connolly, O., Vijayakumar, U.G., Duddy, W.J., and Duguez, S. (2020). Molecular and Cellular Mechanisms Affected in ALS. J. Pers. Med., 10.","DOI":"10.1186\/s13395-020-00238-1"},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"162","DOI":"10.1016\/j.expneurol.2015.09.019","article-title":"Excitotoxicity in ALS: Overstimulation, or overreaction?","volume":"275","author":"King","year":"2016","journal-title":"Exp. Neurol."},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/j.expneurol.2014.05.015","article-title":"Glial cells in amyotrophic lateral sclerosis","volume":"262","author":"Philips","year":"2014","journal-title":"Exp. Neurol."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"253","DOI":"10.1016\/S1474-4422(11)70015-1","article-title":"Neuroinflammation in amyotrophic lateral sclerosis: Role of glial activation in motor neuron disease","volume":"10","author":"Philips","year":"2011","journal-title":"Lancet Neurol."},{"key":"ref_111","doi-asserted-by":"crossref","unstructured":"Bowerman, M., Vincent, T., Scamps, F., Perrin, F.E., Camu, W., and Raoul, C. (2013). Neuroimmunity dynamics and the development of therapeutic strategies for amyotrophic lateral sclerosis. Front. Cell. Neurosci., 7.","DOI":"10.3389\/fncel.2013.00214"},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1016\/S0022-510X(99)00236-1","article-title":"Visualization of defective mitochondrial function in skeletal muscle fibers of patients with sporadic amyotrophic lateral sclerosis","volume":"169","author":"Vielhaber","year":"1999","journal-title":"J. Neurol. Sci."},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"616","DOI":"10.1038\/nrneurol.2011.152","article-title":"Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis","volume":"7","author":"Ferraiuolo","year":"2011","journal-title":"Nat. Rev. Neurol."},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"102","DOI":"10.1016\/j.mito.2021.02.008","article-title":"Mitochondrial dysfunction and traffic jams in amyotrophic lateral sclerosis","volume":"58","author":"Jhanji","year":"2021","journal-title":"Mitochondrion"},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1111\/j.1365-2990.2011.01166.x","article-title":"Review: The role of mitochondria in the pathogenesis of amyotrophic lateral sclerosis","volume":"37","author":"Duffy","year":"2011","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"ref_116","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_117","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_118","doi-asserted-by":"crossref","first-page":"20523","DOI":"10.1073\/pnas.1006869107","article-title":"Deficits in axonal transport precede ALS symptoms in vivo","volume":"107","author":"Bilsland","year":"2010","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_119","doi-asserted-by":"crossref","unstructured":"Zhao, J., Wang, X., Huo, Z., Chen, Y., Liu, J., Zhao, Z., Meng, F., Su, Q., Bao, W., and Zhang, L. (2022). The Impact of Mitochondrial Dysfunction in Amyotrophic Lateral Sclerosis. Cells, 11.","DOI":"10.3390\/cells11132049"},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"113620","DOI":"10.1016\/j.expneurol.2021.113620","article-title":"Impairment of mitochondrial oxidative phosphorylation in skin fibroblasts of SALS and FALS patients is rescued by in vitro treatment with ROS scavengers","volume":"339","author":"Miller","year":"2021","journal-title":"Exp. Neurol."},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1016\/j.neuron.2016.09.015","article-title":"Poly(GR) in C9ORF72-Related ALS\/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons","volume":"92","author":"Lu","year":"2016","journal-title":"Neuron"},{"key":"ref_122","doi-asserted-by":"crossref","unstructured":"Jankovic, M., Novakovic, I., Gamil Anwar Dawod, P., Gamil Anwar Dawod, A., Drinic, A., Abdel Motaleb, F.I., Ducic, S., and Nikolic, D. (2021). Current Concepts on Genetic Aspects of Mitochondrial Dysfunction in Amyotrophic Lateral Sclerosis. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms22189832"},{"key":"ref_123","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1016\/j.neuron.2010.07.019","article-title":"Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS","volume":"67","author":"Israelson","year":"2010","journal-title":"Neuron"},{"key":"ref_124","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1023\/A:1022442629543","article-title":"Hexokinase binding to mitochondria: A basis for proliferative energy metabolism","volume":"29","author":"Bessman","year":"1997","journal-title":"J. Bioenerg. Biomembr."},{"key":"ref_125","doi-asserted-by":"crossref","first-page":"751","DOI":"10.1038\/sj.cdd.4401599","article-title":"The voltage-dependent anion channel-1 modulates apoptotic cell death","volume":"12","author":"Zaid","year":"2005","journal-title":"Cell Death Differ."},{"key":"ref_126","doi-asserted-by":"crossref","first-page":"13482","DOI":"10.1074\/jbc.M708216200","article-title":"Hexokinase-I protection against apoptotic cell death is mediated via interaction with the voltage-dependent anion channel-1: Mapping the site of binding","volume":"283","author":"Zaid","year":"2008","journal-title":"J. Biol. Chem."},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"347","DOI":"10.1042\/bj20031465","article-title":"In self-defence: Hexokinase promotes voltage-dependent anion channel closure and prevents mitochondria-mediated apoptotic cell death","volume":"377","author":"Israelson","year":"2004","journal-title":"Biochem. J."},{"key":"ref_128","doi-asserted-by":"crossref","unstructured":"Pittala, M.G.G., Reina, S., Cubisino, S.A.M., Cucina, A., Formicola, B., Cunsolo, V., Foti, S., Saletti, R., and Messina, A. (2020). Post-Translational Modification Analysis of VDAC1 in ALS-SOD1 Model Cells Reveals Specific Asparagine and Glutamine Deamidation. Antioxidants, 9.","DOI":"10.3390\/antiox9121218"},{"key":"ref_129","doi-asserted-by":"crossref","first-page":"11588","DOI":"10.1523\/JNEUROSCI.5385-12.2013","article-title":"Small peptides against the mutant SOD1\/Bcl-2 toxic mitochondrial complex restore mitochondrial function and cell viability in mutant SOD1-mediated ALS","volume":"33","author":"Tan","year":"2013","journal-title":"J. Neurosci."},{"key":"ref_130","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1038\/nrn2665","article-title":"Mitochondrial membrane permeabilization in neuronal injury","volume":"10","author":"Galluzzi","year":"2009","journal-title":"Nat. Rev. Neurosci."},{"key":"ref_131","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":"ref_132","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1038\/s41420-024-01949-w","article-title":"AAV-mediated upregulation of VDAC1 rescues the mitochondrial respiration and sirtuins expression in a SOD1 mouse model of inherited ALS","volume":"10","author":"Magri","year":"2024","journal-title":"Cell Death Discov."},{"key":"ref_133","doi-asserted-by":"crossref","first-page":"2346","DOI":"10.1016\/j.neuropharm.2012.02.013","article-title":"Olesoxime delays muscle denervation, astrogliosis, microglial activation and motoneuron death in an ALS mouse model","volume":"62","author":"Sunyach","year":"2012","journal-title":"Neuropharmacology"},{"key":"ref_134","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1111\/ene.12344","article-title":"A phase II-III trial of olesoxime in subjects with amyotrophic lateral sclerosis","volume":"21","author":"Lenglet","year":"2014","journal-title":"Eur. J. Neurol."},{"key":"ref_135","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1016\/j.neulet.2018.04.053","article-title":"TDP-43 interacts with mitochondrial proteins critical for mitophagy and mitochondrial dynamics","volume":"678","author":"Davis","year":"2018","journal-title":"Neurosci. Lett."},{"key":"ref_136","doi-asserted-by":"crossref","first-page":"901","DOI":"10.1083\/jcb.200608073","article-title":"Chaperone-mediated coupling of endoplasmic reticulum and mitochondrial Ca2+ channels","volume":"175","author":"Szabadkai","year":"2006","journal-title":"J. Cell Biol."},{"key":"ref_137","doi-asserted-by":"crossref","first-page":"939","DOI":"10.1007\/s00401-022-02494-5","article-title":"PolyGA targets the ER stress-adaptive response by impairing GRP75 function at the MAM in C9ORF72-ALS\/FTD","volume":"144","author":"Pilotto","year":"2022","journal-title":"Acta Neuropathol."},{"key":"ref_138","doi-asserted-by":"crossref","unstructured":"Alvarez-Mora, M.I., Garrabou, G., Barcos, T., Garcia-Garcia, F., Grillo-Risco, R., Peruga, E., Gort, L., Borrego-Ecija, S., Sanchez-Valle, R., and Canto-Santos, J. (2022). Bioenergetic and Autophagic Characterization of Skin Fibroblasts from C9orf72 Patients. Antioxidants, 11.","DOI":"10.3390\/antiox11061129"},{"key":"ref_139","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1038\/s41582-020-0389-4","article-title":"Huntington disease: New insights into molecular pathogenesis and therapeutic opportunities","volume":"16","author":"Tabrizi","year":"2020","journal-title":"Nat. Rev. Neurol."},{"key":"ref_140","doi-asserted-by":"crossref","first-page":"1472","DOI":"10.4103\/1673-5374.360289","article-title":"Mitochondria in Huntington\u2019s disease: Implications in pathogenesis and mitochondrial-targeted therapeutic strategies","volume":"18","author":"Jurcau","year":"2023","journal-title":"Neural Regen. Res."},{"key":"ref_141","doi-asserted-by":"crossref","unstructured":"Karachitos, A., Grobys, D., Kulczynska, K., Sobusiak, A., and Kmita, H. (2016). The Association of VDAC with Cell Viability of PC12 Model of Huntington\u2019s Disease. Front. Oncol., 6.","DOI":"10.3389\/fonc.2016.00238"},{"key":"ref_142","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1016\/j.toxlet.2023.04.011","article-title":"Mitochondrial dysfunction, oxidative stress, ER stress and mitochondria-ER crosstalk alterations in a chemical rat model of Huntington\u2019s disease: Potential benefits of bezafibrate","volume":"381","author":"Brondani","year":"2023","journal-title":"Toxicol. Lett."},{"key":"ref_143","doi-asserted-by":"crossref","first-page":"122362","DOI":"10.1016\/j.lfs.2023.122362","article-title":"Morin suppresses mTORc1\/IRE-1alpha\/JNK and IP3R-VDAC-1 pathways: Crucial mechanisms in apoptosis and mitophagy inhibition in experimental Huntington\u2019s disease, supported by in silico molecular docking simulations","volume":"338","author":"Sheta","year":"2024","journal-title":"Life Sci."},{"key":"ref_144","doi-asserted-by":"crossref","first-page":"345","DOI":"10.3390\/ph3020345","article-title":"Olesoxime (TRO19622): A Novel Mitochondrial-Targeted Neuroprotective Compound","volume":"3","author":"Bordet","year":"2010","journal-title":"Pharmaceuticals"},{"key":"ref_145","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1016\/S1474-4422(17)30085-6","article-title":"Safety and efficacy of olesoxime in patients with type 2 or non-ambulatory type 3 spinal muscular atrophy: A randomised, double-blind, placebo-controlled phase 2 trial","volume":"16","author":"Bertini","year":"2017","journal-title":"Lancet Neurol."},{"key":"ref_146","doi-asserted-by":"crossref","first-page":"959","DOI":"10.1016\/j.nmd.2020.10.008","article-title":"Long-term follow-up of patients with type 2 and non-ambulant type 3 spinal muscular atrophy (SMA) treated with olesoxime in the OLEOS trial","volume":"30","author":"Muntoni","year":"2020","journal-title":"Neuromuscul. Disord."},{"key":"ref_147","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1007\/s10522-022-09960-3","article-title":"Alterations in hippocampal mitochondrial dynamics are associated with neurodegeneration and recognition memory decline in old male mice","volume":"23","author":"Mishra","year":"2022","journal-title":"Biogerontology"},{"key":"ref_148","doi-asserted-by":"crossref","first-page":"4066","DOI":"10.1093\/hmg\/ddp355","article-title":"Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease","volume":"18","author":"Kielar","year":"2009","journal-title":"Hum. Mol. Genet."}],"container-title":["Biomolecules"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2218-273X\/15\/1\/33\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T16:56:42Z","timestamp":1760115402000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2218-273X\/15\/1\/33"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,12,30]]},"references-count":148,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2025,1]]}},"alternative-id":["biom15010033"],"URL":"https:\/\/doi.org\/10.3390\/biom15010033","relation":{},"ISSN":["2218-273X"],"issn-type":[{"value":"2218-273X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,12,30]]}}}