{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T09:26:11Z","timestamp":1790673971972,"version":"4.1.0"},"reference-count":293,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2023,2,24]],"date-time":"2023-02-24T00:00:00Z","timestamp":1677196800000},"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>Huntington\u2019s Disease (HD) is a hereditary neurodegenerative disorder caused by the expansion of a CAG triplet repeat in the HTT gene, resulting in the production of an aberrant huntingtin (Htt) protein. The mutant protein accumulation is responsible for neuronal dysfunction and cell death. This is due to the involvement of oxidative damage, excitotoxicity, inflammation, and mitochondrial impairment. Neurons naturally adapt to bioenergetic alteration and oxidative stress in physiological conditions. However, this dynamic system is compromised when a neurodegenerative disorder occurs, resulting in changes in metabolism, alteration in calcium signaling, and impaired substrates transport. Thus, the aim of this review is to provide an overview of the cell\u2019s answer to the stress induced by HD, focusing on the role of oxidative stress and its balance with the antioxidant system.<\/jats:p>","DOI":"10.3390\/antiox12030571","type":"journal-article","created":{"date-parts":[[2023,2,27]],"date-time":"2023-02-27T03:52:21Z","timestamp":1677469941000},"page":"571","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":37,"title":["Cell Rearrangement and Oxidant\/Antioxidant Imbalance in Huntington\u2019s Disease"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9695-6274","authenticated-orcid":false,"given":"Francesco","family":"D\u2019Egidio","sequence":"first","affiliation":[{"name":"Department of Life, Health and Environmental Sciences, University of L\u2019Aquila, 67100 L\u2019Aquila, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7005-3218","authenticated-orcid":false,"given":"Vanessa","family":"Castelli","sequence":"additional","affiliation":[{"name":"Department of Life, Health and Environmental Sciences, University of L\u2019Aquila, 67100 L\u2019Aquila, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2737-7970","authenticated-orcid":false,"given":"Annamaria","family":"Cimini","sequence":"additional","affiliation":[{"name":"Department of Life, Health and Environmental Sciences, University of L\u2019Aquila, 67100 L\u2019Aquila, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3693-840X","authenticated-orcid":false,"given":"Michele","family":"d\u2019Angelo","sequence":"additional","affiliation":[{"name":"Department of Life, Health and Environmental Sciences, University of L\u2019Aquila, 67100 L\u2019Aquila, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,2,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1016\/S1474-4422(10)70245-3","article-title":"Huntington\u2019s Disease: From Molecular Pathogenesis to Clinical Treatment","volume":"10","author":"Ross","year":"2011","journal-title":"Lancet Neurol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"15005","DOI":"10.1038\/nrdp.2015.5","article-title":"Huntington Disease","volume":"1","author":"Bates","year":"2015","journal-title":"Nat. Rev. Dis. Prim."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"3498","DOI":"10.1073\/pnas.0308679101","article-title":"Venezuelan Kindreds Reveal That Genetic and Environmental Factors Modulate Huntington\u2019s Disease Age of Onset","volume":"101","author":"Wexler","year":"2004","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1038\/ng0494-409","article-title":"Somatic and Gonadal Mosaicism of the Huntington Disease Gene CAG Repeat in Brain and Sperm","volume":"6","author":"Telenius","year":"1994","journal-title":"Nat. Genet."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1097\/00005072-199007000-00002","article-title":"Atrophy of the Hypothalamic Lateral Tuberal Nucleus in Huntington\u2019s Disease","volume":"49","author":"Kremer","year":"1990","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1007\/s004010050408","article-title":"Nerve Cell Loss in the Thalamic Centromedian-Parafascicular Complex in Patients with Huntington\u2019s Disease","volume":"91","author":"Heinsen","year":"1996","journal-title":"Acta Neuropathol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1016\/S0006-8993(99)02320-3","article-title":"Oxidative Stress, Mitochondrial Permeability Transition and Activation of Caspases in Calcium Ionophore A23187-Induced Death of Cultured Striatal Neurons","volume":"857","author":"Castilho","year":"2000","journal-title":"Brain Res."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"846","DOI":"10.1093\/cercor\/bhh185","article-title":"Thalamic Atrophy in Huntington\u2019s Disease Co-Varies with Cognitive Performance: A Morphometric MRI Analysis","volume":"15","author":"Kassubek","year":"2005","journal-title":"Cereb. Cortex"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1093\/hmg\/ddi004","article-title":"Orexin Loss in Huntington\u2019s Disease","volume":"14","author":"Gil","year":"2005","journal-title":"Hum. Mol. Genet."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"5733","DOI":"10.1073\/pnas.85.15.5733","article-title":"Differential Loss of Striatal Projection Neurons in Huntington Disease","volume":"85","author":"Reiner","year":"1988","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1615","DOI":"10.1212\/01.WNL.0000065888.88988.6E","article-title":"Evidence for More Widespread Cerebral Pathology in Early HD: An MRI-Based Morphometric Analysis","volume":"60","author":"Rosas","year":"2003","journal-title":"Neurology"},{"key":"ref_12","first-page":"21","article-title":"Brain Imaging and Cognitive Dysfunctions in Huntington\u2019s Disease","volume":"31","author":"Montoya","year":"2006","journal-title":"J. Psychiatry Neurosci."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1016\/S0140-6736(07)60111-1","article-title":"Huntington\u2019s Disease","volume":"369","author":"Walker","year":"2007","journal-title":"Lancet"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"2670","DOI":"10.1093\/cercor\/bhu065","article-title":"The Corticospinal Tract in Huntington\u2019s Disease","volume":"25","author":"Phillips","year":"2015","journal-title":"Cereb. Cortex"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1586\/14737175.2015.1025383","article-title":"Dopamine and Huntington\u2019s Disease","volume":"15","author":"Schwab","year":"2015","journal-title":"Expert Rev. Neurother."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"3","DOI":"10.3389\/fncir.2018.00003","article-title":"Basal Ganglia Neuromodulation Over Multiple Temporal and Structural Scales\u2014Simulations of Direct Pathway MSNs Investigate the Fast Onset of Dopaminergic Effects and Predict the Role of Kv4.2","volume":"12","author":"Lindroos","year":"2018","journal-title":"Front. Neural Circuits"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1016\/B978-0-444-63425-2.00010-6","article-title":"Chapter 10\u2014The Role of Dopamine in Huntington\u2019s Disease","volume":"Volume 211","author":"Diana","year":"2014","journal-title":"Progress in Brain Research"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1111\/j.1755-5949.2010.00134.x","article-title":"Dopamine and Glutamate in Huntington\u2019s Disease: A Balancing Act","volume":"16","author":"Cepeda","year":"2010","journal-title":"CNS Neurosci. Ther."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1016\/j.bbr.2009.08.028","article-title":"Protective Effect of Hesperidin and Naringin against 3-Nitropropionic Acid Induced Huntington\u2019s like Symptoms in Rats: Possible Role of Nitric Oxide","volume":"206","author":"Kumar","year":"2010","journal-title":"Behav. Brain Res."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"e874387","DOI":"10.1155\/2012\/874387","article-title":"Hippocampal Neurogenesis, Cognitive Deficits and Affective Disorder in Huntington\u2019s Disease","volume":"2012","author":"Ransome","year":"2012","journal-title":"Neural Plast."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"155","DOI":"10.3233\/JHD-120023","article-title":"Cognitive Dysfunction in Huntington\u2019s Disease: Humans, Mouse Models and Molecular Mechanisms","volume":"1","author":"Giralt","year":"2012","journal-title":"J. Huntingt. Dis."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1094","DOI":"10.1093\/brain\/awq047","article-title":"Cell Loss in the Motor and Cingulate Cortex Correlates with Symptomatology in Huntington\u2019s Disease","volume":"133","author":"Thu","year":"2010","journal-title":"Brain"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"1156","DOI":"10.1136\/jnnp-2012-304636","article-title":"Prevalence of Adult Huntington\u2019s Disease in the UK Based on Diagnoses Recorded in General Practice Records","volume":"84","author":"Evans","year":"2013","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1111\/j.1399-0004.2010.01538.x","article-title":"Uptake of Huntington Disease Predictive Testing in a Complete Population","volume":"80","author":"Morrison","year":"2011","journal-title":"Clin. Genet."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1016\/B978-0-12-801893-4.00003-1","article-title":"Chapter 3\u2014Epidemiology of Huntington Disease","volume":"144","author":"Feigin","year":"2017","journal-title":"Handbook of Clinical Neurology"},{"key":"ref_26","doi-asserted-by":"crossref","unstructured":"Bates, G., Tabrizi, S., and Jones, L. (2014). Huntington\u2019s Disease, Oxford University Press.","DOI":"10.1093\/med\/9780199929146.001.0001"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1707","DOI":"10.1002\/mds.25266","article-title":"Prevalence Estimates of Huntington Disease in Caucasian Populations Are Gross Underestimates","volume":"27","author":"Morrison","year":"2012","journal-title":"Mov. Disord."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1136\/jnnp.2004.036806","article-title":"Incidence and Mutation Rates of Huntington\u2019s Disease in Spain: Experience of 9 Years of Direct Genetic Testing","volume":"76","author":"Moreno","year":"2005","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"198","DOI":"10.1034\/j.1399-0004.2001.600305.x","article-title":"High Incidence Rate and Absent Family Histories in One Quarter of Patients Newly Diagnosed with Huntington Disease in British Columbia","volume":"60","author":"Almqvist","year":"2001","journal-title":"Clin. Genet."},{"key":"ref_30","first-page":"193","article-title":"The Prevalence of Huntington\u2019s Chorea in South Africa","volume":"58","author":"Hayden","year":"1980","journal-title":"S. Afr. Med. J."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1016\/0092-8674(93)90585-E","article-title":"A Novel Gene Containing a Trinucleotide Repeat That Is Expanded and Unstable on Huntington\u2019s Disease Chromosomes. The Huntington\u2019s Disease Collaborative Research Group","volume":"72","author":"MacDonald","year":"1993","journal-title":"Cell"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1016\/j.neuron.2012.05.024","article-title":"Network Organization of the Huntingtin Proteomic Interactome in Mammalian Brain","volume":"75","author":"Shirasaki","year":"2012","journal-title":"Neuron"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1006\/exnr.1998.6832","article-title":"Wild-Type and Mutant Huntingtins Function in Vesicle Trafficking in the Secretory and Endocytic Pathways","volume":"152","author":"Velier","year":"1998","journal-title":"Exp. Neurol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1093\/hmg\/ddi014","article-title":"Transcriptional Dysregulation in Striatal Projection- and Interneurons in a Mouse Model of Huntington\u2019s Disease: Neuronal Selectivity and Potential Neuroprotective Role of HAP1","volume":"14","author":"Zucker","year":"2005","journal-title":"Hum. Mol. Genet."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"1259","DOI":"10.1093\/hmg\/9.9.1259","article-title":"Decreased Expression of Striatal Signaling Genes in a Mouse Model of Huntington\u2019s Disease","volume":"9","author":"Strand","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1385\/MN:28:3:259","article-title":"Wild-Type Huntingtin Plays a Role in Brain Development and Neuronal Survival","volume":"28","author":"Reiner","year":"2003","journal-title":"Mol. Neurobiol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/978-3-319-71779-1_1","article-title":"Clinical Features of Huntington\u2019s Disease","volume":"Volume 1049","author":"Ghosh","year":"2018","journal-title":"Polyglutamine Disorders. Advances in Experimental Medicine and Biology"},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"1990","DOI":"10.1126\/science.277.5334.1990","article-title":"Aggregation of Huntingtin in Neuronal Intranuclear Inclusions and Dystrophic Neurites in Brain","volume":"277","author":"DiFiglia","year":"1997","journal-title":"Science"},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1111\/j.1471-4159.2005.03348.x","article-title":"Purification of Neuronal Inclusions of Patients with Huntington\u2019s Disease Reveals a Broad Range of N-Terminal Fragments of Expanded Huntingtin and Insoluble Polymers","volume":"95","author":"Hoffner","year":"2005","journal-title":"J. Neurochem."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"783","DOI":"10.1093\/hmg\/7.5.783","article-title":"Truncated N-Terminal Fragments of Huntingtin with Expanded Glutamine Repeats Form Nuclear and Cytoplasmic Aggregates in Cell Culture","volume":"7","author":"Cooper","year":"1998","journal-title":"Hum. Mol. Genet."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1038\/nature02998","article-title":"Inclusion Body Formation Reduces Levels of Mutant Huntingtin and the Risk of Neuronal Death","volume":"431","author":"Arrasate","year":"2004","journal-title":"Nature"},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"11402","DOI":"10.1073\/pnas.0503634102","article-title":"Absence of Behavioral Abnormalities and Neurodegeneration In Vivo despite Widespread Neuronal Huntingtin Inclusions","volume":"102","author":"Slow","year":"2005","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"2894","DOI":"10.1016\/j.bpj.2012.04.050","article-title":"Fibrillar \u03b1-Synuclein and Huntingtin Exon 1 Assemblies Are Toxic to the Cells","volume":"102","author":"Pieri","year":"2012","journal-title":"Biophys. J."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"16017","DOI":"10.1074\/jbc.M111.252577","article-title":"Identification of Novel Potentially Toxic Oligomers Formed In Vitro from Mammalian-Derived Expanded Huntingtin Exon-1 Protein","volume":"287","author":"Nucifora","year":"2012","journal-title":"J. Biol. Chem."},{"key":"ref_45","doi-asserted-by":"crossref","unstructured":"Lajoie, P., and Snapp, E.L. (2010). Formation and Toxicity of Soluble Polyglutamine Oligomers in Living Cells. PLoS ONE, 5.","DOI":"10.1371\/journal.pone.0015245"},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"332","DOI":"10.1038\/nsmb1215","article-title":"A Toxic Monomeric Conformer of the Polyglutamine Protein","volume":"14","author":"Nagai","year":"2007","journal-title":"Nat. Struct. Mol. Biol."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"925","DOI":"10.1038\/nchembio.694","article-title":"Identifying Polyglutamine Protein Species in Situ That Best Predict Neurodegeneration","volume":"7","author":"Miller","year":"2011","journal-title":"Nat. Chem. Biol."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"895","DOI":"10.1038\/srep00895","article-title":"Cellular Inclusion Bodies of Mutant Huntingtin Exon 1 Obscure Small Fibrillar Aggregate Species","volume":"2","author":"Sahl","year":"2012","journal-title":"Sci. Rep."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"2366","DOI":"10.1073\/pnas.1221891110","article-title":"Aberrant Splicing of HTT Generates the Pathogenic Exon 1 Protein in Huntington Disease","volume":"110","author":"Sathasivam","year":"2013","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1006\/exnr.1998.7005","article-title":"Huntington\u2019s Disease Intranuclear Inclusions Contain Truncated, Ubiquitinated Huntingtin Protein","volume":"156","author":"Sieradzan","year":"1999","journal-title":"Exp. Neurol."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"2753","DOI":"10.1038\/ncomms3753","article-title":"Soluble Forms of PolyQ-Expanded Huntingtin Rather than Large Aggregates Cause Endoplasmic Reticulum Stress","volume":"4","author":"Leitman","year":"2013","journal-title":"Nat. Commun."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1093\/hmg\/ddm311","article-title":"Soluble Polyglutamine Oligomers Formed Prior to Inclusion Body Formation Are Cytotoxic","volume":"17","author":"Takahashi","year":"2008","journal-title":"Hum. Mol. Genet."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"2738","DOI":"10.1093\/hmg\/ddn175","article-title":"Accumulation of N-Terminal Mutant Huntingtin in Mouse and Monkey Models Implicated as a Pathogenic Mechanism in Huntington\u2019s Disease","volume":"17","author":"Wang","year":"2008","journal-title":"Hum. Mol. Genet."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1016\/j.nbd.2011.12.032","article-title":"Induced Pluripotent Stem Cell Lines from Huntington\u2019s Disease Mice Undergo Neuronal Differentiation While Showing Alterations in the Lysosomal Pathway","volume":"46","author":"Castiglioni","year":"2012","journal-title":"Neurobiol. Dis."},{"key":"ref_55","doi-asserted-by":"crossref","unstructured":"The HD iPSC Consortium (2012). HD iPSC Consortium Induced Pluripotent Stem Cells from Patients with Huntington\u2019s Disease Show CAG-Repeat-Expansion-Associated Phenotypes. Cell Stem Cell, 11, 264\u2013278.","DOI":"10.1016\/j.stem.2012.04.027"},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"2905","DOI":"10.1093\/hmg\/11.23.2905","article-title":"Aggregated Polyglutamine Peptides Delivered to Nuclei Are Toxic to Mammalian Cells","volume":"11","author":"Yang","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"19180","DOI":"10.1038\/srep19180","article-title":"\u03b1-Synuclein and Huntingtin Exon 1 Amyloid Fibrils Bind Laterally to the Cellular Membrane","volume":"6","author":"Monsellier","year":"2016","journal-title":"Sci. Rep."},{"key":"ref_58","first-page":"3678","article-title":"Transfer of Polyglutamine Aggregates in Neuronal Cells Occurs in Tunneling Nanotubes","volume":"126","author":"Costanzo","year":"2013","journal-title":"J. Cell Sci."},{"key":"ref_59","doi-asserted-by":"crossref","unstructured":"Herrera, F., Tenreiro, S., Miller-Fleming, L., and Outeiro, T.F. (2011). Visualization of Cell-to-Cell Transmission of Mutant Huntingtin Oligomers. PLoS Curr., 3.","DOI":"10.1371\/currents.RRN1210"},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"E5427","DOI":"10.1073\/pnas.1516217112","article-title":"Transcellular Spreading of Huntingtin Aggregates in the Drosophila Brain","volume":"112","author":"Babcock","year":"2015","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"6768","DOI":"10.1038\/ncomms7768","article-title":"Prion-like Transmission of Neuronal Huntingtin Aggregates to Phagocytic Glia in the Drosophila Brain","volume":"6","author":"Pearce","year":"2015","journal-title":"Nat. Commun."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"1064","DOI":"10.1038\/nn.3761","article-title":"Transneuronal Propagation of Mutant Huntingtin Contributes to Non-Cell Autonomous Pathology in Neurons","volume":"17","author":"Rieker","year":"2014","journal-title":"Nat. Neurosci."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"14777","DOI":"10.1074\/jbc.M109.093708","article-title":"Mutant Huntingtin Fragments Form Oligomers in a Polyglutamine Length-Dependent Manner In Vitro and In Vivo","volume":"285","author":"Legleiter","year":"2010","journal-title":"J. Biol. Chem."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"675","DOI":"10.1016\/j.molcel.2018.07.032","article-title":"MHTT Seeding Activity: A Marker of Disease Progression and Neurotoxicity in Models of Huntington\u2019s Disease","volume":"71","author":"Ast","year":"2018","journal-title":"Mol. Cell"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1002\/ana.24174","article-title":"Mutant Huntingtin Is Present in Neuronal Grafts in Huntington Disease Patients","volume":"76","author":"Cicchetti","year":"2014","journal-title":"Ann. Neurol."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1038\/nrm.2016.147","article-title":"The Nuclear Pore Complex: Understanding Its Function through Structural Insight","volume":"18","author":"Beck","year":"2017","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"ref_67","first-page":"82","article-title":"The RanGTP Pathway: From Nucleo-Cytoplasmic Transport to Spindle Assembly and Beyond","volume":"3","author":"Cavazza","year":"2015","journal-title":"Front. Cell Dev. Biol."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"E177","DOI":"10.1038\/ncb0702-e177","article-title":"The Ran GTPase as a Marker of Chromosome Position in Spindle Formation and Nuclear Envelope Assembly","volume":"4","author":"Hetzer","year":"2002","journal-title":"Nat. Cell Biol."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"1134","DOI":"10.1016\/j.celrep.2015.04.030","article-title":"Quantitative Interaction Proteomics of Neurodegenerative Disease Proteins","volume":"11","author":"Hosp","year":"2015","journal-title":"Cell Rep."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"604","DOI":"10.1126\/science.6254144","article-title":"DNA Methylation and Gene Function","volume":"210","author":"Razin","year":"1980","journal-title":"Science"},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1038\/nn.3607","article-title":"Distribution, Recognition and Regulation of Non-CpG Methylation in the Adult Mammalian Brain","volume":"17","author":"Guo","year":"2014","journal-title":"Nat. Neurosci."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"19176","DOI":"10.1073\/pnas.0606373103","article-title":"ESET\/SETDB1 Gene Expression and Histone H3 (K9) Trimethylation in Huntington\u2019s Disease","volume":"103","author":"Ryu","year":"2006","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"1164","DOI":"10.1093\/hmg\/ddm064","article-title":"Modulation of Nucleosome Dynamics in Huntington\u2019s Disease","volume":"16","author":"Stack","year":"2007","journal-title":"Hum. Mol. Genet."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1016\/j.neulet.2015.10.060","article-title":"DNA Methylation in Huntington\u2019s Disease: Implications for Transgenerational Effects","volume":"625","author":"Thomas","year":"2016","journal-title":"Neurosci. Lett."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"E56","DOI":"10.1073\/pnas.1415195112","article-title":"HDAC Inhibition Imparts Beneficial Transgenerational Effects in Huntington\u2019s Disease Mice via Altered DNA and Histone Methylation","volume":"112","author":"Jia","year":"2015","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"2354","DOI":"10.1073\/pnas.1221292110","article-title":"Extensive Changes in DNA Methylation Are Associated with Expression of Mutant Huntingtin","volume":"110","author":"Ng","year":"2013","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"1485","DOI":"10.18632\/aging.101005","article-title":"Huntington\u2019s Disease Accelerates Epigenetic Aging of Human Brain and Disrupts DNA Methylation Levels","volume":"8","author":"Horvath","year":"2016","journal-title":"Aging"},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"434","DOI":"10.1101\/gr.103101.109","article-title":"Human Aging-Associated DNA Hypermethylation Occurs Preferentially at Bivalent Chromatin Domains","volume":"20","author":"Rakyan","year":"2010","journal-title":"Genome Res."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"440","DOI":"10.1101\/gr.103606.109","article-title":"Age-Dependent DNA Methylation of Genes That Are Suppressed in Stem Cells Is a Hallmark of Cancer","volume":"20","author":"Teschendorff","year":"2010","journal-title":"Genome Res."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"260","DOI":"10.1016\/j.ajhg.2011.12.020","article-title":"DNA Methylation Signatures in Development and Aging of the Human Prefrontal Cortex","volume":"90","author":"Numata","year":"2012","journal-title":"Am. J. Hum. Genet."},{"key":"ref_81","doi-asserted-by":"crossref","unstructured":"Johansson, A., Enroth, S., and Gyllensten, U. (2013). Continuous Aging of the Human DNA Methylome throughout the Human Lifespan. PLoS ONE, 8.","DOI":"10.1371\/journal.pone.0067378"},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"4529","DOI":"10.1038\/s41467-020-18255-5","article-title":"DNA Methylation Study of Huntington\u2019s Disease and Motor Progression in Patients and in Animal Models","volume":"11","author":"Lu","year":"2020","journal-title":"Nat. Commun."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"1407","DOI":"10.1093\/hmg\/ddh162","article-title":"Mutant Huntingtin Directly Increases Susceptibility of Mitochondria to the Calcium-Induced Permeability Transition and Cytochrome c Release","volume":"13","author":"Choo","year":"2004","journal-title":"Hum. Mol. Genet."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"731","DOI":"10.1038\/nn884","article-title":"Early Mitochondrial Calcium Defects in Huntington\u2019s Disease Are a Direct Effect of Polyglutamines","volume":"5","author":"Panov","year":"2002","journal-title":"Nat. Neurosci."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"822","DOI":"10.1038\/nn.3721","article-title":"Inhibition of Mitochondrial Protein Import by Mutant Huntingtin","volume":"17","author":"Yano","year":"2014","journal-title":"Nat. Neurosci."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"16593","DOI":"10.1073\/pnas.1904101116","article-title":"Mutant Huntingtin Disrupts Mitochondrial Proteostasis by Interacting with TIM23","volume":"116","author":"Yablonska","year":"2019","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1007\/s10863-010-9286-7","article-title":"The Interrelationship between Mitochondrial Dysfunction and Transcriptional Dysregulation in Huntington Disease","volume":"42","author":"Jin","year":"2010","journal-title":"J. Bioenerg. Biomembr."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"1478","DOI":"10.1016\/j.freeradbiomed.2012.06.008","article-title":"Mitochondrial DNA Damage Is Associated with Reduced Mitochondrial Bioenergetics in Huntington\u2019s Disease","volume":"53","author":"Siddiqui","year":"2012","journal-title":"Free Radic. Biol. Med."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"531","DOI":"10.1023\/B:NERE.0000014824.04728.dd","article-title":"The Energetics of Huntington\u2019s Disease","volume":"29","author":"Browne","year":"2004","journal-title":"Neurochem. Res."},{"key":"ref_90","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_91","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1016\/j.freeradbiomed.2013.04.016","article-title":"PGC-1\u03b1, Mitochondrial Dysfunction, and Huntington\u2019s Disease","volume":"62","author":"Johri","year":"2013","journal-title":"Free Radic. Biol. Med."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1002\/ana.410390317","article-title":"Mitochondrial Defect in Huntington\u2019s Disease Caudate Nucleus","volume":"39","author":"Gu","year":"1996","journal-title":"Ann. Neurol."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"646","DOI":"10.1002\/ana.410410514","article-title":"Oxidative Damage and Metabolic Dysfunction in Huntington\u2019s Disease: Selective Vulnerability of the Basal Ganglia","volume":"41","author":"Browne","year":"1997","journal-title":"Ann. Neurol."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1016\/j.cell.2006.09.015","article-title":"Transcriptional Repression of PGC-1alpha by Mutant Huntingtin Leads to Mitochondrial Dysfunction and Neurodegeneration","volume":"127","author":"Cui","year":"2006","journal-title":"Cell"},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"103","DOI":"10.3389\/fncel.2019.00103","article-title":"Mitochondrial Dysfunction in Huntington\u2019s Disease; Interplay Between HSF1, P53 and PGC-1\u03b1 Transcription Factors","volume":"13","author":"Intihar","year":"2019","journal-title":"Front. Cell Neurosci."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"2783","DOI":"10.1523\/JNEUROSCI.0106-08.2008","article-title":"N-Terminal Mutant Huntingtin Associates with Mitochondria and Impairs Mitochondrial Trafficking","volume":"28","author":"Orr","year":"2008","journal-title":"J. Neurosci."},{"key":"ref_97","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_98","doi-asserted-by":"crossref","first-page":"1438","DOI":"10.1093\/hmg\/ddr024","article-title":"Abnormal Mitochondrial Dynamics, Mitochondrial Loss and Mutant Huntingtin Oligomers in Huntington\u2019s Disease: Implications for Selective Neuronal Damage","volume":"20","author":"Shirendeb","year":"2011","journal-title":"Hum. Mol. Genet."},{"key":"ref_99","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_100","doi-asserted-by":"crossref","first-page":"1073","DOI":"10.1128\/MCB.01434-12","article-title":"Regulation of Feedback between Protein Kinase A and the Proteasome System Worsens Huntington\u2019s Disease","volume":"33","author":"Lin","year":"2013","journal-title":"Mol. Cell Biol."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"963","DOI":"10.1016\/j.drudis.2014.02.014","article-title":"The Many Faces of Autophagy Dysfunction in Huntington\u2019s Disease: From Mechanism to Therapy","volume":"19","author":"Cortes","year":"2014","journal-title":"Drug Discov. Today"},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"3308","DOI":"10.1101\/gad.1673408","article-title":"Impaired ERAD and ER Stress Are Early and Specific Events in Polyglutamine Toxicity","volume":"22","author":"Duennwald","year":"2008","journal-title":"Genes Dev."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1016\/j.molcel.2017.06.017","article-title":"The Unfolded Protein Response and Cell Fate Control","volume":"69","author":"Hetz","year":"2018","journal-title":"Mol. Cell"},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1016\/j.tcb.2013.06.005","article-title":"IRE1: ER Stress Sensor and Cell Fate Executor","volume":"23","author":"Chen","year":"2013","journal-title":"Trends Cell Biol."},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"881","DOI":"10.1016\/S0092-8674(01)00611-0","article-title":"XBP1 MRNA Is Induced by ATF6 and Spliced by IRE1 in Response to ER Stress to Produce a Highly Active Transcription Factor","volume":"107","author":"Yoshida","year":"2001","journal-title":"Cell"},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1016\/S1534-5807(02)00203-4","article-title":"ER Stress Regulation of ATF6 Localization by Dissociation of BiP\/GRP78 Binding and Unmasking of Golgi Localization Signals","volume":"3","author":"Shen","year":"2002","journal-title":"Dev. Cell"},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1038\/nrm3270","article-title":"The Unfolded Protein Response: Controlling Cell Fate Decisions under ER Stress and Beyond","volume":"13","author":"Hetz","year":"2012","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"7198","DOI":"10.1128\/MCB.23.20.7198-7209.2003","article-title":"Nrf2 Is a Direct PERK Substrate and Effector of PERK-Dependent Cell Survival","volume":"23","author":"Cullinan","year":"2003","journal-title":"Mol. Cell Biol."},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1038\/sj.cdd.4401373","article-title":"Roles of CHOP\/GADD153 in Endoplasmic Reticulum Stress","volume":"11","author":"Oyadomari","year":"2004","journal-title":"Cell Death Differ."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"3460","DOI":"10.1016\/j.bbamcr.2013.06.028","article-title":"ER Stress-Induced Cell Death Mechanisms","volume":"1833","author":"Sano","year":"2013","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1016\/j.brainresbull.2017.08.007","article-title":"PERK as a Hub of Multiple Pathogenic Pathways Leading to Memory Deficits and Neurodegeneration in Alzheimer\u2019s Disease","volume":"141","author":"Ohno","year":"2018","journal-title":"Brain Res. Bull."},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1080\/20964471.2018.1472392","article-title":"Mapping Landslide Susceptibility and Types Using Random Forest","volume":"2","author":"Taalab","year":"2018","journal-title":"Big Earth Data"},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"950","DOI":"10.1016\/j.yexcr.2007.12.025","article-title":"Inhibition of Endoplasmic Reticulum Stress Counteracts Neuronal Cell Death and Protein Aggregation Caused by N-Terminal Mutant Huntingtin Proteins","volume":"314","author":"Reijonen","year":"2008","journal-title":"Exp. Cell Res."},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"440","DOI":"10.3858\/emm.2009.41.6.049","article-title":"Induction of Unfolded Protein Response during Neuronal Induction of Rat Bone Marrow Stromal Cells and Mouse Embryonic Stem Cells","volume":"41","author":"Cho","year":"2009","journal-title":"Exp. Mol. Med."},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"11318","DOI":"10.1074\/jbc.M807620200","article-title":"SCAMP5 Links Endoplasmic Reticulum Stress to the Accumulation of Expanded Polyglutamine Protein Aggregates via Endocytosis Inhibition","volume":"284","author":"Noh","year":"2009","journal-title":"J. Biol. Chem."},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"18167","DOI":"10.1074\/jbc.M109.018325","article-title":"Rrs1 Is Involved in Endoplasmic Reticulum Stress Response in Huntington Disease","volume":"284","author":"Carnemolla","year":"2009","journal-title":"J. Biol. Chem."},{"key":"ref_117","doi-asserted-by":"crossref","first-page":"2245","DOI":"10.1093\/hmg\/dds040","article-title":"Targeting the UPR Transcription Factor XBP1 Protects against Huntington\u2019s Disease through the Regulation of FoxO1 and Autophagy","volume":"21","author":"Vidal","year":"2012","journal-title":"Hum. Mol. Genet."},{"key":"ref_118","doi-asserted-by":"crossref","unstructured":"Leitman, J., Barak, B., Benyair, R., Shenkman, M., Ashery, U., Hartl, F.U., and Lederkremer, G.Z. (2014). ER Stress-Induced EIF2-Alpha Phosphorylation Underlies Sensitivity of Striatal Neurons to Pathogenic Huntingtin. PLoS ONE, 9.","DOI":"10.1371\/journal.pone.0090803"},{"key":"ref_119","doi-asserted-by":"crossref","unstructured":"Yang, H., Liu, C., Zhong, Y., Luo, S., Monteiro, M.J., and Fang, S. (2010). Huntingtin Interacts with the Cue Domain of Gp78 and Inhibits Gp78 Binding to Ubiquitin and P97\/VCP. PLoS ONE, 5.","DOI":"10.1371\/journal.pone.0008905"},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"5928","DOI":"10.1093\/hmg\/ddu317","article-title":"Ubiquitin-Specific Protease-14 Reduces Cellular Aggregates and Protects against Mutant Huntingtin-Induced Cell Degeneration: Involvement of the Proteasome and ER Stress-Activated Kinase IRE1\u03b1","volume":"23","author":"Hyrskyluoto","year":"2014","journal-title":"Hum. Mol. Genet."},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"839","DOI":"10.1007\/s00401-017-1770-2","article-title":"The Neuroprotective Transcription Factor ATF5 Is Decreased and Sequestered into Polyglutamine Inclusions in Huntington\u2019s Disease","volume":"134","author":"Lucas","year":"2017","journal-title":"Acta Neuropathol."},{"key":"ref_122","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1016\/j.brainresbull.2009.07.010","article-title":"Role of Mitochondrial Dysfunction in the Pathogenesis of Huntington\u2019s Disease","volume":"80","author":"Quintanilla","year":"2009","journal-title":"Brain Res. Bull."},{"key":"ref_123","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1016\/j.bbadis.2009.07.012","article-title":"Mitochondria in Huntington\u2019s Disease","volume":"1802","author":"Damiano","year":"2010","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_124","doi-asserted-by":"crossref","first-page":"342","DOI":"10.5142\/jgr.2012.36.4.342","article-title":"Effects of Panax Ginseng in Neurodegenerative Diseases","volume":"36","author":"Cho","year":"2012","journal-title":"J. Ginseng. Res."},{"key":"ref_125","doi-asserted-by":"crossref","first-page":"5780","DOI":"10.1074\/jbc.M704704200","article-title":"Calcium Homeostasis and Mitochondrial Dysfunction in Striatal Neurons of Huntington Disease","volume":"283","author":"Lim","year":"2008","journal-title":"J. Biol. Chem."},{"key":"ref_126","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1002\/1531-8249(199901)45:1<25::AID-ART6>3.0.CO;2-E","article-title":"Biochemical Abnormalities and Excitotoxicity in Huntington\u2019s Disease Brain","volume":"45","author":"Tabrizi","year":"1999","journal-title":"Ann. Neurol."},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1007\/s00702-009-0328-4","article-title":"Abnormal Morphology of Peripheral Cell Tissues from Patients with Huntington Disease","volume":"117","author":"Squitieri","year":"2010","journal-title":"J. Neural Transm."},{"key":"ref_128","doi-asserted-by":"crossref","first-page":"664","DOI":"10.1016\/j.bbadis.2011.11.014","article-title":"Antioxidants in Huntington\u2019s Disease","volume":"1822","author":"Johri","year":"2012","journal-title":"Biochim. Biophys. Acta (BBA)\u2014Mol. Basis Dis."},{"key":"ref_129","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_130","doi-asserted-by":"crossref","first-page":"578","DOI":"10.1016\/S0166-2236(02)02270-1","article-title":"Regulation of AMPA Receptors during Synaptic Plasticity","volume":"25","author":"Song","year":"2002","journal-title":"Trends Neurosci."},{"key":"ref_131","doi-asserted-by":"crossref","first-page":"518","DOI":"10.1007\/s12640-017-9766-1","article-title":"Huntington\u2019s Disease and Mitochondria","volume":"32","author":"Ghaedi","year":"2017","journal-title":"Neurotox. Res."},{"key":"ref_132","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1007\/978-3-319-71779-1_3","article-title":"Mitochondrial Dysfunction in Huntington\u2019s Disease","volume":"1049","author":"Carmo","year":"2018","journal-title":"Adv. Exp. Med. Biol."},{"key":"ref_133","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1111\/j.1749-6632.2010.05629.x","article-title":"Dynamic Regulation of Mitochondrial Fission through Modification of the Dynamin-Related Protein Drp1","volume":"1201","author":"Chang","year":"2010","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_134","doi-asserted-by":"crossref","first-page":"1127","DOI":"10.1038\/embor.2013.168","article-title":"Loss of Iron Triggers PINK1\/Parkin-Independent Mitophagy","volume":"14","author":"Allen","year":"2013","journal-title":"EMBO Rep."},{"key":"ref_135","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1038\/cdd.2014.139","article-title":"AMBRA1 Is Able to Induce Mitophagy via LC3 Binding, Regardless of PARKIN and P62\/SQSTM1","volume":"22","author":"Strappazzon","year":"2015","journal-title":"Cell Death Differ."},{"key":"ref_136","doi-asserted-by":"crossref","first-page":"1293","DOI":"10.1523\/JNEUROSCI.1870-13.2014","article-title":"The Regulation of Autophagosome Dynamics by Huntingtin and HAP1 Is Disrupted by Expression of Mutant Huntingtin, Leading to Defective Cargo Degradation","volume":"34","author":"Wong","year":"2014","journal-title":"J. Neurosci."},{"key":"ref_137","doi-asserted-by":"crossref","first-page":"262","DOI":"10.1038\/ncb3101","article-title":"Huntingtin Functions as a Scaffold for Selective Macroautophagy","volume":"17","author":"Rui","year":"2015","journal-title":"Nat. Cell Biol."},{"key":"ref_138","doi-asserted-by":"crossref","first-page":"685","DOI":"10.1016\/j.molcel.2015.10.009","article-title":"Measuring In Vivo Mitophagy","volume":"60","author":"Sun","year":"2015","journal-title":"Mol. Cell"},{"key":"ref_139","doi-asserted-by":"crossref","first-page":"329","DOI":"10.3389\/fnmol.2018.00329","article-title":"A Mitochondria-Associated Oxidative Stress Perspective on Huntington\u2019s Disease","volume":"11","author":"Zheng","year":"2018","journal-title":"Front. Mol. Neurosci."},{"key":"ref_140","doi-asserted-by":"crossref","first-page":"C1424","DOI":"10.1152\/ajpcell.1996.271.5.C1424","article-title":"Nitric Oxide, Superoxide, and Peroxynitrite: The Good, the Bad, and Ugly","volume":"271","author":"Beckman","year":"1996","journal-title":"Am. J. Physiol.-Cell Physiol."},{"key":"ref_141","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1016\/j.ejmech.2015.04.040","article-title":"The Role of Antioxidants in the Chemistry of Oxidative Stress: A Review","volume":"97","author":"Pisoschi","year":"2015","journal-title":"Eur. J. Med. Chem."},{"key":"ref_142","doi-asserted-by":"crossref","first-page":"472","DOI":"10.3390\/biom5020472","article-title":"Biological Activities of Reactive Oxygen and Nitrogen Species: Oxidative Stress versus Signal Transduction","volume":"5","author":"Weidinger","year":"2015","journal-title":"Biomolecules"},{"key":"ref_143","doi-asserted-by":"crossref","unstructured":"Collin, F. (2019). Chemical Basis of Reactive Oxygen Species Reactivity and Involvement in Neurodegenerative Diseases. Int. J. Mol. Sci., 20.","DOI":"10.3390\/ijms20102407"},{"key":"ref_144","doi-asserted-by":"crossref","first-page":"2325","DOI":"10.1351\/pac200779122325","article-title":"Fenton Chemistry in Biology and Medicine","volume":"79","author":"Prousek","year":"2007","journal-title":"Pure Appl. Chem."},{"key":"ref_145","doi-asserted-by":"crossref","first-page":"158","DOI":"10.1016\/j.molcel.2012.09.025","article-title":"Physiological Roles of Mitochondrial Reactive Oxygen Species","volume":"48","author":"Sena","year":"2012","journal-title":"Mol. Cell"},{"key":"ref_146","doi-asserted-by":"crossref","first-page":"325","DOI":"10.5607\/en.2015.24.4.325","article-title":"The Role of Oxidative Stress in Neurodegenerative Diseases","volume":"24","author":"Kim","year":"2015","journal-title":"Exp. Neurobiol."},{"key":"ref_147","doi-asserted-by":"crossref","first-page":"1343","DOI":"10.1089\/ars.2007.1957","article-title":"Redox Regulation of Cell Survival","volume":"10","author":"Trachootham","year":"2008","journal-title":"Antioxid. Redox Signal."},{"key":"ref_148","doi-asserted-by":"crossref","first-page":"1363","DOI":"10.1016\/j.bbadis.2011.12.001","article-title":"Role of Peroxisomes in ROS\/RNS-Metabolism: Implications for Human Disease","volume":"1822","author":"Fransen","year":"2012","journal-title":"Biochim. Biophys. Acta Mol. Basis Dis."},{"key":"ref_149","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1042\/BJ20111752","article-title":"Cell Signalling by Reactive Lipid Species: New Concepts and Molecular Mechanisms","volume":"442","author":"Higdon","year":"2012","journal-title":"Biochem. J."},{"key":"ref_150","doi-asserted-by":"crossref","first-page":"1976","DOI":"10.1194\/jlr.R066597","article-title":"Oxidative Stress and Lipotoxicity","volume":"57","author":"Hauck","year":"2016","journal-title":"J. Lipid Res."},{"key":"ref_151","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1016\/j.biopha.2015.07.025","article-title":"Oxidative Stress, Mitochondrial Dysfunction and Neurodegenerative Diseases; a Mechanistic Insight","volume":"74","author":"Bhat","year":"2015","journal-title":"Biomed. Pharmacother."},{"key":"ref_152","doi-asserted-by":"crossref","first-page":"1407","DOI":"10.1038\/nm.2056","article-title":"Balance between Synaptic versus Extrasynaptic NMDA Receptor Activity Influences Inclusions and Neurotoxicity of Mutant Huntingtin","volume":"15","author":"Okamoto","year":"2009","journal-title":"Nat. Med."},{"key":"ref_153","doi-asserted-by":"crossref","first-page":"346","DOI":"10.1111\/jnc.14723","article-title":"Alterations in Synaptic Function and Plasticity in Huntington Disease","volume":"150","author":"Sepers","year":"2019","journal-title":"J. Neurochem."},{"key":"ref_154","doi-asserted-by":"crossref","first-page":"562","DOI":"10.1016\/j.cardiores.2005.01.026","article-title":"PGC-1\u03b1 Regulates the Mitochondrial Antioxidant Defense System in Vascular Endothelial Cells","volume":"66","author":"Valle","year":"2005","journal-title":"Cardiovasc. Res."},{"key":"ref_155","doi-asserted-by":"crossref","first-page":"3474","DOI":"10.1093\/hmg\/dds178","article-title":"Transducer of Regulated CREB-Binding Proteins (TORCs) Transcription and Function Is Impaired in Huntington\u2019s Disease","volume":"21","author":"Chaturvedi","year":"2012","journal-title":"Hum. Mol. Genet."},{"key":"ref_156","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1002\/emmm.201000084","article-title":"Inhibition of Transglutaminase 2 Mitigates Transcriptional Dysregulation in Models of Huntington Disease","volume":"2","author":"McConoughey","year":"2010","journal-title":"EMBO Mol. Med."},{"key":"ref_157","doi-asserted-by":"crossref","first-page":"68","DOI":"10.3389\/fnmol.2019.00068","article-title":"Impaired Redox Signaling in Huntington\u2019s Disease: Therapeutic Implications","volume":"12","author":"Paul","year":"2019","journal-title":"Front. Mol. Neurosci."},{"key":"ref_158","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1016\/S0304-3940(99)00578-9","article-title":"Oxidative Damage to Mitochondrial DNA in Huntington\u2019s Disease Parietal Cortex","volume":"272","author":"Polidori","year":"1999","journal-title":"Neurosci. Lett."},{"key":"ref_159","doi-asserted-by":"crossref","first-page":"1110","DOI":"10.1016\/j.dnarep.2008.03.012","article-title":"Mitochondrial DNA Damage and Repair in Neurodegenerative Disorders","volume":"7","author":"Yang","year":"2008","journal-title":"DNA Repair"},{"key":"ref_160","doi-asserted-by":"crossref","first-page":"34","DOI":"10.32607\/20758251-2017-9-2-34-46","article-title":"Huntington\u2019s Disease: Calcium Dyshomeostasis and Pathology Models","volume":"9","author":"Kolobkova","year":"2017","journal-title":"Acta Nat."},{"key":"ref_161","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1016\/j.nbd.2011.07.001","article-title":"What Have We Learned from Gene Expression Profiles in Huntington\u2019s Disease?","volume":"45","author":"Seredenina","year":"2012","journal-title":"Neurobiol. Dis."},{"key":"ref_162","doi-asserted-by":"crossref","first-page":"573","DOI":"10.1083\/jcb.201110093","article-title":"Indirect Inhibition of 26S Proteasome Activity in a Cellular Model of Huntington\u2019s Disease","volume":"196","author":"Hipp","year":"2012","journal-title":"J. Cell Biol."},{"key":"ref_163","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1038\/nn.2528","article-title":"Cargo Recognition Failure Is Responsible for Inefficient Autophagy in Huntington\u2019s Disease","volume":"13","author":"Talloczy","year":"2010","journal-title":"Nat. Neurosci."},{"key":"ref_164","doi-asserted-by":"crossref","first-page":"1655","DOI":"10.1007\/s11064-007-9352-7","article-title":"Myelin Breakdown and Iron Changes in Huntington\u2019s Disease: Pathogenesis and Treatment Implications","volume":"32","author":"Bartzokis","year":"2007","journal-title":"Neurochem. Res."},{"key":"ref_165","doi-asserted-by":"crossref","first-page":"S94","DOI":"10.1002\/ana.410320716","article-title":"Alterations in Levels of Iron, Ferritin, and Other Trace Metals in Neurodegenerative Diseases Affecting the Basal Ganglia","volume":"32","author":"Dexter","year":"1992","journal-title":"Ann. Neurol."},{"key":"ref_166","doi-asserted-by":"crossref","first-page":"132","DOI":"10.3389\/fnmol.2019.00132","article-title":"Neuronal Cells Rearrangement during Aging and Neurodegenerative Disease: Metabolism, Oxidative Stress and Organelles Dynamic","volume":"12","author":"Castelli","year":"2019","journal-title":"Front. Mol. Neurosci."},{"key":"ref_167","doi-asserted-by":"crossref","first-page":"2017","DOI":"10.1007\/s00429-017-1383-5","article-title":"Human Astrocytes: Structure and Functions in the Healthy Brain","volume":"222","author":"Vasile","year":"2017","journal-title":"Brain Struct. Funct."},{"key":"ref_168","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1017\/S1740925X12000166","article-title":"Physiological Function of Microglia","volume":"7","author":"Wake","year":"2011","journal-title":"Neuron Glia Biol."},{"key":"ref_169","doi-asserted-by":"crossref","first-page":"3865","DOI":"10.1002\/cne.24040","article-title":"The Search for True Numbers of Neurons and Glial Cells in the Human Brain: A Review of 150 Years of Cell Counting","volume":"524","author":"Bahney","year":"2016","journal-title":"J. Comp. Neurol."},{"key":"ref_170","first-page":"278","article-title":"Elusive Roles for Reactive Astrocytes in Neurodegenerative Diseases","volume":"9","author":"Escartin","year":"2015","journal-title":"Front. Cell. Neurosci."},{"key":"ref_171","doi-asserted-by":"crossref","first-page":"281","DOI":"10.31887\/DCNS.2009.11.3\/mbelanger","article-title":"The Role of Astroglia in Neuroprotection","volume":"11","author":"Magistretti","year":"2009","journal-title":"Dialogues Clin. Neurosci."},{"key":"ref_172","doi-asserted-by":"crossref","first-page":"a020628","DOI":"10.1101\/cshperspect.a020628","article-title":"Astrocytes in Neurodegenerative Disease","volume":"7","author":"Phatnani","year":"2015","journal-title":"Cold Spring Harb. Perspect. Biol."},{"key":"ref_173","doi-asserted-by":"crossref","first-page":"e6501031","DOI":"10.1155\/2018\/6501031","article-title":"Mechanisms of Endogenous Neuroprotective Effects of Astrocytes in Brain Injury","volume":"2018","author":"Bylicky","year":"2018","journal-title":"Oxidative Med. Cell. Longev."},{"key":"ref_174","doi-asserted-by":"crossref","first-page":"e484613","DOI":"10.1155\/2013\/484613","article-title":"Role of Redox Signaling in Neuroinflammation and Neurodegenerative Diseases","volume":"2013","author":"Hsieh","year":"2013","journal-title":"BioMed Res. Int."},{"key":"ref_175","doi-asserted-by":"crossref","first-page":"258","DOI":"10.3389\/fnmol.2019.00258","article-title":"The Role of Microglia and Astrocytes in Huntington\u2019s Disease","volume":"12","author":"Palpagama","year":"2019","journal-title":"Front. Mol. Neurosci."},{"key":"ref_176","doi-asserted-by":"crossref","first-page":"422","DOI":"10.1016\/j.tins.2017.05.002","article-title":"Unravelling and Exploiting Astrocyte Dysfunction in Huntington\u2019s Disease","volume":"40","author":"Khakh","year":"2017","journal-title":"Trends Neurosci."},{"key":"ref_177","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1007\/978-981-13-9913-8_14","article-title":"Astrocytes in Huntington\u2019s Disease","volume":"1175","author":"Gray","year":"2019","journal-title":"Adv. Exp. Med. Biol."},{"key":"ref_178","doi-asserted-by":"crossref","first-page":"577380","DOI":"10.1016\/j.jneuroim.2020.577380","article-title":"Inflammation in Huntington\u2019s Disease: A Few New Twists on an Old Tale","volume":"348","author":"Santos","year":"2020","journal-title":"J. Neuroimmunol."},{"key":"ref_179","doi-asserted-by":"crossref","first-page":"540","DOI":"10.1002\/jnr.20180","article-title":"Redox Regulation of Glial Inflammatory Response to Lipopolysaccharide and Interferon\u03b3","volume":"77","author":"Pawate","year":"2004","journal-title":"J. Neurosci. Res."},{"key":"ref_180","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1002\/jbt.10058","article-title":"Diabetes, Oxidative Stress, and Antioxidants: A Review","volume":"17","author":"Maritim","year":"2003","journal-title":"J. Biochem. Mol. Toxicol."},{"key":"ref_181","doi-asserted-by":"crossref","first-page":"872","DOI":"10.1016\/S0899-9007(02)00916-4","article-title":"Free Radicals, Antioxidants, and Nutrition","volume":"18","author":"Fang","year":"2002","journal-title":"Nutrition"},{"key":"ref_182","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1016\/S0955-0674(03)00002-4","article-title":"Oxidant Signals and Oxidative Stress","volume":"15","author":"Finkel","year":"2003","journal-title":"Curr. Opin. Cell Biol."},{"key":"ref_183","doi-asserted-by":"crossref","first-page":"18515","DOI":"10.1074\/jbc.272.30.18515","article-title":"Superoxide Anion Radical (O2-.), Superoxide Dismutases, and Related Matters","volume":"272","author":"Fridovich","year":"1997","journal-title":"J. Biol. Chem."},{"key":"ref_184","doi-asserted-by":"crossref","first-page":"1006","DOI":"10.1139\/o84-129","article-title":"Catalase: An Old Enzyme with a New Role?","volume":"62","author":"Percy","year":"1984","journal-title":"Can. J. Biochem. Cell Biol."},{"key":"ref_185","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1104\/pp.106.077073","article-title":"Reactive Species and Antioxidants. Redox Biology Is a Fundamental Theme of Aerobic Life","volume":"141","author":"Halliwell","year":"2006","journal-title":"Plant Physiol."},{"key":"ref_186","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1016\/j.tips.2010.12.002","article-title":"Free Radicals and Antioxidants\u2014Quo Vadis?","volume":"32","author":"Halliwell","year":"2011","journal-title":"Trends Pharmacol. Sci."},{"key":"ref_187","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1124\/pr.57.2.4","article-title":"The Blood-Brain Barrier\/Neurovascular Unit in Health and Disease","volume":"57","author":"Hawkins","year":"2005","journal-title":"Pharmacol. Rev."},{"key":"ref_188","doi-asserted-by":"crossref","first-page":"1263","DOI":"10.1097\/00001756-200307010-00015","article-title":"Ascorbate Treatment Attenuates the Huntington Behavioral Phenotype in Mice","volume":"14","author":"Rebec","year":"2003","journal-title":"Neuroreport"},{"key":"ref_189","doi-asserted-by":"crossref","first-page":"1085","DOI":"10.1016\/j.freeradbiomed.2015.09.024","article-title":"Beyond the Redox Imbalance: Oxidative Stress Contributes to an Impaired GLUT3 Modulation in Huntington\u2019s Disease","volume":"89","author":"Moll","year":"2015","journal-title":"Free Radic. Biol. Med."},{"key":"ref_190","doi-asserted-by":"crossref","first-page":"2917","DOI":"10.1038\/ncomms3917","article-title":"A Failure in Energy Metabolism and Antioxidant Uptake Precede Symptoms of Huntington\u2019s Disease in Mice","volume":"4","author":"Esparza","year":"2013","journal-title":"Nat. Commun."},{"key":"ref_191","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1046\/j.1471-4159.2003.01857.x","article-title":"Protective Effects of the Antioxidant Selenium on Quinolinic Acid-induced Neurotoxicity in Rats: In Vitro and In Vivo Studies","volume":"86","author":"Ali","year":"2004","journal-title":"J. Neurochem."},{"key":"ref_192","doi-asserted-by":"crossref","first-page":"214","DOI":"10.1007\/s12640-012-9336-5","article-title":"Organoselenium Bis Selenide Attenuates 3-Nitropropionic Acid-Induced Neurotoxicity in Rats","volume":"23","author":"Bortolatto","year":"2013","journal-title":"Neurotox. Res."},{"key":"ref_193","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1016\/j.nbd.2014.06.022","article-title":"Altered Selenium Status in Huntington\u2019s Disease: Neuroprotection by Selenite in the N171-82Q Mouse Model","volume":"71","author":"Lu","year":"2014","journal-title":"Neurobiol. Dis."},{"key":"ref_194","first-page":"424","article-title":"The molecular bases of Huntington\u2019s disease: The role played by oxidative stress","volume":"49","author":"Tasset","year":"2009","journal-title":"Rev. Neurol."},{"key":"ref_195","doi-asserted-by":"crossref","first-page":"5602","DOI":"10.1021\/jm200605a","article-title":"Important Role of Oxidative Stress Biomarkers in Huntington\u2019s Disease","volume":"54","year":"2011","journal-title":"J. Med. Chem."},{"key":"ref_196","first-page":"388","article-title":"Essential Fatty Acid-Rich Diets Protect against Striatal Oxidative Damage Induced by Quinolinic Acid in Rats","volume":"20","author":"Montes","year":"2016","journal-title":"Nutr. Neurosci."},{"key":"ref_197","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1016\/0952-3278(92)90062-N","article-title":"Use of Gamma-Linolenic Acid in the Treatment of Schizophrenia and Tardive Dyskinesia","volume":"46","author":"Vaddadi","year":"1992","journal-title":"Prostaglandins Leukot. Essent. Fat. Acids"},{"key":"ref_198","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1097\/00001756-200201210-00011","article-title":"A Randomised, Placebo-Controlled, Double Blind Study of Treatment of Huntington\u2019s Disease with Unsaturated Fatty Acids","volume":"13","author":"Vaddadi","year":"2002","journal-title":"Neuroreport"},{"key":"ref_199","doi-asserted-by":"crossref","first-page":"4389","DOI":"10.1523\/JNEUROSCI.20-12-04389.2000","article-title":"Neuroprotective Effects of Creatine in a Transgenic Mouse Model of Huntington\u2019s Disease","volume":"20","author":"Ferrante","year":"2000","journal-title":"J. Neurosci."},{"key":"ref_200","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1006\/nbdi.2001.0406","article-title":"Creatine Increase Survival and Delays Motor Symptoms in a Transgenic Animal Model of Huntington\u2019s Disease","volume":"8","author":"Andreassen","year":"2001","journal-title":"Neurobiol. Dis."},{"key":"ref_201","doi-asserted-by":"crossref","first-page":"1833","DOI":"10.1097\/00001756-200006260-00007","article-title":"Creatine Reduces 3-Nitropropionic-Acid-Induced Cognitive and Motor Abnormalities in Rats","volume":"11","author":"Shear","year":"2000","journal-title":"Neuroreport"},{"key":"ref_202","doi-asserted-by":"crossref","first-page":"250","DOI":"10.1212\/01.wnl.0000194318.74946.b6","article-title":"Creatine in Huntington Disease Is Safe, Tolerable, Bioavailable in Brain and Reduces Serum 8OH2\u2032dG","volume":"66","author":"Hersch","year":"2006","journal-title":"Neurology"},{"key":"ref_203","doi-asserted-by":"crossref","first-page":"1305","DOI":"10.1007\/s00726-011-0851-0","article-title":"Neuroprotective Effects of Creatine","volume":"40","author":"Beal","year":"2011","journal-title":"Amino Acids"},{"key":"ref_204","doi-asserted-by":"crossref","first-page":"594","DOI":"10.1212\/WNL.0000000000004209","article-title":"The CREST-E Study of Creatine for Huntington Disease: A Randomized Controlled Trial","volume":"89","author":"Hersch","year":"2017","journal-title":"Neurology"},{"key":"ref_205","doi-asserted-by":"crossref","first-page":"1592","DOI":"10.1523\/JNEUROSCI.22-05-01592.2002","article-title":"Therapeutic Effects of Coenzyme Q10 and Remacemide in Transgenic Mouse Models of Huntington\u2019s Disease","volume":"22","author":"Ferrante","year":"2002","journal-title":"J. Neurosci."},{"key":"ref_206","doi-asserted-by":"crossref","first-page":"1427","DOI":"10.1111\/j.1471-4159.2009.06074.x","article-title":"Combination Therapy with Coenzyme Q10 and Creatine Produces Additive Neuroprotective Effects in Models of Parkinson\u2019s and Huntington\u2019s Diseases","volume":"109","author":"Yang","year":"2009","journal-title":"J. Neurochem."},{"key":"ref_207","doi-asserted-by":"crossref","unstructured":"Huntington Study Group Pre2CARE Investigators (2010). Safety and Tolerability of High-dosage Coenzyme Q10 in Huntington\u2019s Disease and Healthy Subjects. Mov. Disord., 25, 1924\u20131928. Available online: https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/10.1002\/mds.22408.","DOI":"10.1002\/mds.22408"},{"key":"ref_208","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1016\/0014-4886(90)90005-D","article-title":"Idebenone Attenuates Neuronal Degeneration Induced by Intrastriatal Injection of Excitotoxins","volume":"108","author":"Miyamoto","year":"1990","journal-title":"Exp. Neurol."},{"key":"ref_209","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1002\/mds.870110510","article-title":"A Controlled Trial of Idebenone in Huntington\u2019s Disease","volume":"11","author":"Ranen","year":"1996","journal-title":"Mov. Disord."},{"key":"ref_210","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1159\/000443896","article-title":"Neuroprotective Activity of Curcumin in Combination with Piperine against Quinolinic Acid Induced Neurodegeneration in Rats","volume":"97","author":"Singh","year":"2016","journal-title":"Pharmacology"},{"key":"ref_211","doi-asserted-by":"crossref","first-page":"18736","DOI":"10.1038\/srep18736","article-title":"Curcumin Modulates Cell Death and Is Protective in Huntington\u2019s Disease Model","volume":"6","author":"Chongtham","year":"2016","journal-title":"Sci. Rep."},{"key":"ref_212","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1177\/15353702211046927","article-title":"Management of Altered Metabolic Activity in Drosophila Model of Huntington\u2019s Disease by Curcumin","volume":"247","author":"Aditi","year":"2022","journal-title":"Exp. Biol. Med."},{"key":"ref_213","first-page":"4012","article-title":"Curcumin Dietary Supplementation Ameliorates Disease Phenotype in an Animal Model of Huntington\u2019s Disease","volume":"28","author":"Elifani","year":"2019","journal-title":"Hum. Mol. Genet."},{"key":"ref_214","doi-asserted-by":"crossref","first-page":"95","DOI":"10.2478\/v10134-010-0022-y","article-title":"Potential Application of Grape Derived Polyphenols in Huntington\u2019s Disease","volume":"1","author":"Wang","year":"2010","journal-title":"Transl. Neurosci."},{"key":"ref_215","doi-asserted-by":"crossref","first-page":"711","DOI":"10.1016\/j.lfs.2009.10.001","article-title":"Lycopene Modulates Nitric Oxide Pathways against 3-Nitropropionic Acid-Induced Neurotoxicity","volume":"85","author":"Kumar","year":"2009","journal-title":"Life Sci."},{"key":"ref_216","doi-asserted-by":"crossref","first-page":"2522","DOI":"10.1016\/j.fct.2009.07.011","article-title":"Effect of Lycopene and Epigallocatechin-3-Gallate against 3-Nitropropionic Acid Induced Cognitive Dysfunction and Glutathione Depletion in Rat: A Novel Nitric Oxide Mechanism","volume":"47","author":"Kumar","year":"2009","journal-title":"Food Chem. Toxicol."},{"key":"ref_217","doi-asserted-by":"crossref","first-page":"186","DOI":"10.5455\/jice.20140903012921","article-title":"Combination of Lycopene, Quercetin and Poloxamer 188 Alleviates Anxiety and Depression in 3-Nitropropionic Acid-Induced Huntington\u2019s Disease in Rats","volume":"3","author":"Jain","year":"2014","journal-title":"J. Intercult. Ethnopharmacol."},{"key":"ref_218","doi-asserted-by":"crossref","first-page":"252","DOI":"10.1111\/j.1600-079X.2004.00163.x","article-title":"Protective Effect of Melatonin on 3-Nitropropionic Acid-Induced Oxidative Stress in Synaptosomes in an Animal Model of Huntington\u2019s Disease","volume":"37","author":"Montilla","year":"2004","journal-title":"J. Pineal Res."},{"key":"ref_219","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1016\/j.ejphar.2013.01.007","article-title":"Correlations between Behavioural and Oxidative Parameters in a Rat Quinolinic Acid Model of Huntington\u2019s Disease: Protective Effect of Melatonin","volume":"701","year":"2013","journal-title":"Eur. J. Pharmacol."},{"key":"ref_220","doi-asserted-by":"crossref","first-page":"631","DOI":"10.1016\/0306-4522(96)00155-8","article-title":"Protective Effect of Melatonin against Hippocampal Dna Damage Induced by Intraperitoneal Administration of Kainate to Rats","volume":"73","author":"Uz","year":"1996","journal-title":"Neuroscience"},{"key":"ref_221","doi-asserted-by":"crossref","first-page":"1709","DOI":"10.1046\/j.1471-4159.2000.0751709.x","article-title":"Effect of Exogenous and Endogenous Antioxidants on 3-Nitropionic Acid-Induced In Vivo Oxidative Stress and Striatal Lesions: Insights into Huntington\u2019s Disease","volume":"75","author":"Fontaine","year":"2000","journal-title":"J. Neurochem."},{"key":"ref_222","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1159\/000334273","article-title":"N-Acetylcysteine Reverses Mitochondrial Dysfunctions and Behavioral Abnormalities in 3-Nitropropionic Acid-Induced Huntington\u2019s Disease","volume":"9","author":"Sandhir","year":"2012","journal-title":"Neurodegener. Dis."},{"key":"ref_223","first-page":"2923","article-title":"N-Acetylcysteine Modulates Glutamatergic Dysfunction and Depressive Behavior in Huntington\u2019s Disease","volume":"25","author":"Wright","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"ref_224","unstructured":"(2023, January 13). Wjpps|ABSTRACT. Available online: https:\/\/www.wjpps.com\/Wjpps_controller\/abstract_id\/2546."},{"key":"ref_225","doi-asserted-by":"crossref","first-page":"471","DOI":"10.1007\/s11011-016-9929-4","article-title":"Effect of Rutin against a Mitochondrial Toxin, 3-Nitropropionicacid Induced Biochemical, Behavioral and Histological Alterations-a Pilot Study on Huntington\u2019s Disease Model in Rats","volume":"32","author":"Suganya","year":"2017","journal-title":"Metab. Brain Dis."},{"key":"ref_226","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1006\/exnr.2001.7755","article-title":"A Bile Acid Protects against Motor and Cognitive Deficits and Reduces Striatal Degeneration in the 3-Nitropropionic Acid Model of Huntington\u2019s Disease","volume":"171","author":"Keene","year":"2001","journal-title":"Exp. Neurol."},{"key":"ref_227","doi-asserted-by":"crossref","first-page":"10671","DOI":"10.1073\/pnas.162362299","article-title":"Tauroursodeoxycholic Acid, a Bile Acid, Is Neuroprotective in a Transgenic Animal Model of Huntington\u2019s Disease","volume":"99","author":"Keene","year":"2002","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_228","doi-asserted-by":"crossref","first-page":"377","DOI":"10.3109\/01480541003642050","article-title":"Possible Nitric Oxide Modulation in Protective Effect of FK-506 against 3-Nitropropionic Acid-Induced Behavioral, Oxidative, Neurochemical, and Mitochondrial Alterations in Rat Brain","volume":"33","author":"Kumar","year":"2010","journal-title":"Drug Chem. Toxicol."},{"key":"ref_229","doi-asserted-by":"crossref","first-page":"600","DOI":"10.1016\/j.neuint.2011.04.009","article-title":"FK506 Ameliorates Cell Death Features in Huntington\u2019s Disease Striatal Cell Models","volume":"59","author":"Rosenstock","year":"2011","journal-title":"Neurochem. Int."},{"key":"ref_230","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1016\/j.freeradbiomed.2010.03.017","article-title":"Triterpenoids CDDO-Ethyl Amide and CDDO-Trifluoroethyl Amide Improve the Behavioral Phenotype and Brain Pathology in a Transgenic Mouse Model of Huntington\u2019s Disease","volume":"49","author":"Stack","year":"2010","journal-title":"Free Radic. Biol. Med."},{"key":"ref_231","doi-asserted-by":"crossref","first-page":"1137","DOI":"10.1016\/j.celrep.2012.10.001","article-title":"Targeting of XJB-5-131 to Mitochondria Suppresses Oxidative DNA Damage and Motor Decline in a Mouse Model of Huntington\u2019s Disease","volume":"2","author":"Xun","year":"2012","journal-title":"Cell Rep."},{"key":"ref_232","doi-asserted-by":"crossref","first-page":"1792","DOI":"10.1093\/hmg\/ddw051","article-title":"Mitochondrial Targeting of XJB-5-131 Attenuates or Improves Pathophysiology in HdhQ150 Animals with Well-Developed Disease Phenotypes","volume":"25","author":"Polyzos","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"ref_233","doi-asserted-by":"crossref","unstructured":"Polyzos, A.A., Wood, N.I., Williams, P., Wipf, P., Morton, A.J., and McMurray, C.T. (2018). XJB-5-131-Mediated Improvement in Physiology and Behaviour of the R6\/2 Mouse Model of Huntington\u2019s Disease Is Age- and Sex- Dependent. PLoS ONE, 13.","DOI":"10.1371\/journal.pone.0194580"},{"key":"ref_234","doi-asserted-by":"crossref","unstructured":"Colle, D., Santos, D.B., Moreira, E.L.G., Hartwig, J.M., dos Santos, A.A., Zimmermann, L.T., Hort, M.A., and Farina, M. (2013). Probucol Increases Striatal Glutathione Peroxidase Activity and Protects against 3-Nitropropionic Acid-Induced Pro-Oxidative Damage in Rats. PLoS ONE, 8.","DOI":"10.1371\/journal.pone.0067658"},{"key":"ref_235","first-page":"4056383","article-title":"Antidepressant Effects of Probucol on Early-Symptomatic YAC128 Transgenic Mice for Huntington\u2019s Disease","volume":"2018","author":"Wink","year":"2018","journal-title":"Neural Plast."},{"key":"ref_236","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1046\/j.1471-4159.2003.t01-1-01868.x","article-title":"Increased Survival and Neuroprotective Effects of BN82451 in a Transgenic Mouse Model of Huntington\u2019s Disease","volume":"86","author":"Klivenyi","year":"2003","journal-title":"J. Neurochem."},{"key":"ref_237","doi-asserted-by":"crossref","first-page":"863","DOI":"10.1016\/j.cell.2011.05.020","article-title":"Kynurenine 3-Monooxygenase Inhibition in Blood Ameliorates Neurodegeneration","volume":"145","author":"Zwilling","year":"2011","journal-title":"Cell"},{"key":"ref_238","doi-asserted-by":"crossref","first-page":"e2349","DOI":"10.1002\/brb3.2349","article-title":"Zinc Deficiency Correlates with Severity of Diabetic Polyneuropathy","volume":"11","author":"Hussein","year":"2021","journal-title":"Brain Behav."},{"key":"ref_239","unstructured":"(2022, July 15). Office of Dietary Supplements\u2014Zinc, Available online: https:\/\/ods.od.nih.gov\/factsheets\/Zinc-HealthProfessional\/."},{"key":"ref_240","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1111\/j.1471-4159.2009.06151.x","article-title":"A Metabolic Switch in Brain: Glucose and Lactate Metabolism Modulation by Ascorbic Acid","volume":"110","author":"Castro","year":"2009","journal-title":"J. Neurochem."},{"key":"ref_241","doi-asserted-by":"crossref","first-page":"815","DOI":"10.1046\/j.1471-4159.2001.00461.x","article-title":"High-Affinity Sodium-Vitamin C Co-Transporters (SVCT) Expression in Embryonic Mouse Neurons","volume":"78","author":"Castro","year":"2001","journal-title":"J. Neurochem."},{"key":"ref_242","doi-asserted-by":"crossref","first-page":"773","DOI":"10.1111\/j.1471-4159.2007.04631.x","article-title":"Intracellular Ascorbic Acid Inhibits Transport of Glucose by Neurons, but Not by Astrocytes","volume":"102","author":"Castro","year":"2007","journal-title":"J. Neurochem."},{"key":"ref_243","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1006\/abbi.1997.0473","article-title":"Protection and Recycling of \u03b1-Tocopherol in Human Erythrocytes by Intracellular Ascorbic Acid","volume":"349","author":"May","year":"1998","journal-title":"Arch. Biochem. Biophys."},{"key":"ref_244","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1097\/00001756-199011000-00004","article-title":"Ascorbic Acid Protects Neurons from Injury Induced by Glutamate and NMDA","volume":"1","author":"Majewska","year":"1990","journal-title":"Neuroreport"},{"key":"ref_245","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.jinorgbio.2015.09.003","article-title":"Importance of Selenium and Selenoprotein for Brain Function: From Antioxidant Protection to Neuronal Signalling","volume":"153","author":"Solovyev","year":"2015","journal-title":"J. Inorg. Biochem."},{"key":"ref_246","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1159\/000253483","article-title":"Olive Oil and Cognition: Results from the Three-City Study","volume":"28","author":"Berr","year":"2009","journal-title":"Dement. Geriatr. Cogn. Disord."},{"key":"ref_247","doi-asserted-by":"crossref","first-page":"1189","DOI":"10.1016\/j.exger.2004.05.002","article-title":"Dietary Fat Type (Virgin Olive vs. Sunflower Oils) Affects Age-Related Changes in DNA Double-Strand-Breaks, Antioxidant Capacity and Blood Lipids in Rats","volume":"39","author":"Quiles","year":"2004","journal-title":"Exp. Gerontol."},{"key":"ref_248","first-page":"375","article-title":"Bioavailability and Antioxidant Effects of Olive Oil Phenolic Compounds in Humans: A Review","volume":"43","author":"Khymenetz","year":"2007","journal-title":"Ann. Ist. Super Sanita"},{"key":"ref_249","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1016\/j.neulet.2008.09.022","article-title":"Neuroprotective Effect of Hydroxytyrosol and Hydroxytyrosol Acetate in Rat Brain Slices Subjected to Hypoxia\u2013Reoxygenation","volume":"446","author":"Navas","year":"2008","journal-title":"Neurosci. Lett."},{"key":"ref_250","doi-asserted-by":"crossref","first-page":"333","DOI":"10.7326\/0003-4819-145-5-200609050-00006","article-title":"The Effect of Polyphenols in Olive Oil on Heart Disease Risk Factors: A Randomized Trial","volume":"145","author":"Covas","year":"2006","journal-title":"Ann. Intern. Med."},{"key":"ref_251","doi-asserted-by":"crossref","first-page":"1119","DOI":"10.1007\/s11745-003-1169-7","article-title":"Comparison of the Effects of Dietary Sunflower Oil and Virgin Olive Oil on Rat Exocrine Pancreatic Secretion In Vivo","volume":"38","author":"Yago","year":"2003","journal-title":"Lipids"},{"key":"ref_252","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1096\/fasebj.13.2.411","article-title":"(R)-Alpha-Lipoic Acid-Supplemented Old Rats Have Improved Mitochondrial Function, Decreased Oxidative Damage, and Increased Metabolic Rate","volume":"13","author":"Hagen","year":"1999","journal-title":"FASEB J."},{"key":"ref_253","doi-asserted-by":"crossref","first-page":"1379","DOI":"10.1093\/hmg\/ddi147","article-title":"Loss of Wild-Type Huntingtin Influences Motor Dysfunction and Survival in the YAC128 Mouse Model of Huntington Disease","volume":"14","author":"Pearson","year":"2005","journal-title":"Hum. Mol. Genet."},{"key":"ref_254","doi-asserted-by":"crossref","first-page":"137","DOI":"10.2174\/2211556011201020137","article-title":"Therapeutic Strategies for Huntingtons Disease: From the Bench to the Clinic","volume":"1","author":"Brocardo","year":"2012","journal-title":"Curr. Psychopharmacol."},{"key":"ref_255","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1186\/1550-2783-11-11","article-title":"Effects of Creatine Supplementation Associated with Resistance Training on Oxidative Stress in Different Tissues of Rats","volume":"11","author":"Stefani","year":"2014","journal-title":"J. Int. Soc. Sport. Nutr."},{"key":"ref_256","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1002\/ana.1028","article-title":"Potential for Creatine and Other Therapies Targeting Cellular Energy Dysfunction in Neurological Disorders","volume":"49","author":"Tarnopolsky","year":"2001","journal-title":"Ann. Neurol."},{"key":"ref_257","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1212\/01.WNL.0000070186.97463.A7","article-title":"Creatine Therapy for Huntington\u2019s Disease: Clinical and MRS Findings in a 1-Year Pilot Study","volume":"61","author":"Tabrizi","year":"2003","journal-title":"Neurology"},{"key":"ref_258","doi-asserted-by":"crossref","first-page":"1655","DOI":"10.1212\/01.WNL.0000160388.96242.77","article-title":"High-Dose Creatine Therapy for Huntington Disease: A 2-Year Clinical and MRS Study","volume":"64","author":"Tabrizi","year":"2005","journal-title":"Neurology"},{"key":"ref_259","doi-asserted-by":"crossref","unstructured":"Huntington Study Group (2001). A Randomized, Placebo-Controlled Trial of Coenzyme Q10 and Remacemide in Huntington\u2019s Disease. Neurology, 57, 397\u2013404.","DOI":"10.1212\/WNL.57.3.397"},{"key":"ref_260","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1002\/mds.870110317","article-title":"Assessment of Coenzyme Q10 Tolerability in Huntington\u2019s Disease","volume":"11","author":"Feigin","year":"1996","journal-title":"Mov. Disord."},{"key":"ref_261","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1002\/mds.870110310","article-title":"A Controlled Trial of Remacemide Hydrochloride in Huntington\u2019s Disease","volume":"11","author":"Kieburtz","year":"1996","journal-title":"Mov. Disord."},{"key":"ref_262","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1016\/S0304-3940(01)02326-6","article-title":"Coenzyme Q10 and Remacemide Hydrochloride Ameliorate Motor Deficits in a Huntington\u2019s Disease Transgenic Mouse Model","volume":"315","author":"Schilling","year":"2001","journal-title":"Neurosci. Lett."},{"key":"ref_263","doi-asserted-by":"crossref","first-page":"1400","DOI":"10.1016\/j.amjcard.2007.06.030","article-title":"Effect of Coenzyme Q(10) Supplementation on Simvastatin-Induced Myalgia","volume":"100","author":"Young","year":"2007","journal-title":"Am. J. Cardiol."},{"key":"ref_264","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1007\/s12640-018-9989-9","article-title":"Protective Effects of Antioxidants in Huntington\u2019s Disease: An Extensive Review","volume":"35","author":"Essa","year":"2019","journal-title":"Neurotox. Res."},{"key":"ref_265","unstructured":"Second Affiliated Hospital, School of Medicine, Zhejiang University (2021). Non-Randomized Control Clinical Trial to Evaluate the Efficacy and Safety of Symptomatic Drug Therapy for Mild to Moderate Huntington\u2019s Disease Patients."},{"key":"ref_266","doi-asserted-by":"crossref","unstructured":"D\u2019Egidio, F., Lombardozzi, G., Kacem Ben Haj M\u2019Barek, H.E., Mastroiacovo, G., Alfonsetti, M., and Cimini, A. (2022). The Influence of Dietary Supplementations on Neuropathic Pain. Life, 12.","DOI":"10.3390\/life12081125"},{"key":"ref_267","doi-asserted-by":"crossref","first-page":"579","DOI":"10.1586\/epr.10.69","article-title":"Polyphenolic Compounds for Treating Neurodegenerative Disorders Involving Protein Misfolding","volume":"7","author":"Ho","year":"2010","journal-title":"Expert Rev. Proteom."},{"key":"ref_268","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1016\/j.tox.2008.11.005","article-title":"Melatonin Pretreatment Attenuates 2-Bromopropane-Induced Testicular Toxicity in Rats","volume":"256","author":"Huang","year":"2009","journal-title":"Toxicology"},{"key":"ref_269","unstructured":"Stimming, E.F. (2022). Melatonin for Huntington\u2019s Disease (HD) Gene Carriers with HD Related Sleep Disturbance\u2014A Pilot Study."},{"key":"ref_270","doi-asserted-by":"crossref","first-page":"1281","DOI":"10.1211\/jpp\/61.10.0003","article-title":"Boswellia Resin: From Religious Ceremonies to Medical Uses: A Review of In-Vitro, In-Vivo and Clinical Trials","volume":"61","author":"Moussaieff","year":"2009","journal-title":"J. Pharm. Pharmacol."},{"key":"ref_271","unstructured":"Peterson, B., and Nguyen, H. (2022). StatPearls, StatPearls Publishing."},{"key":"ref_272","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1016\/S0149-7634(96)00027-9","article-title":"3-Nitropropionic Acid Animal Model and Huntington\u2019s Disease","volume":"21","author":"Borlongan","year":"1997","journal-title":"Neurosci. Biobehav. Rev."},{"key":"ref_273","unstructured":"Loy, C. (2022). A Randomised Controlled Trial, Of N-Acetyl Cysteine (NAC), for Premanifest Huntingtin Gene Expansion Carriers."},{"key":"ref_274","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1016\/j.foodchem.2005.03.019","article-title":"Antioxidant Flavonol Glycosides in Mulberry (Morus Alba L.) Leaves Isolated Based on LDL Antioxidant Activity","volume":"97","author":"Katsube","year":"2006","journal-title":"Food Chem."},{"key":"ref_275","doi-asserted-by":"crossref","first-page":"1709","DOI":"10.1016\/j.biortech.2004.12.025","article-title":"Optimal Recovery of High-Purity Rutin Crystals from the Whole Plant of Fagopyrum Esculentum Moench (Buckwheat) by Extraction, Fractionation, and Recrystallization","volume":"96","author":"Kim","year":"2005","journal-title":"Bioresour. Technol."},{"key":"ref_276","first-page":"84","article-title":"Effect of rutin and esculamine on models of aseptic inflammation","volume":"49","author":"Aleksandrov","year":"1986","journal-title":"Farmakol. Toksikol."},{"key":"ref_277","doi-asserted-by":"crossref","first-page":"687","DOI":"10.1016\/S0024-3205(97)01164-8","article-title":"Oral Administration of Rutoside Can Ameliorate Inflammatory Bowel Disease in Rats","volume":"62","author":"Cruz","year":"1998","journal-title":"Life Sci."},{"key":"ref_278","doi-asserted-by":"crossref","first-page":"471","DOI":"10.1046\/j.1365-2567.2000.00045.x","article-title":"Naturally Occurring Polyphenolic Antioxidants Modulate IgE-Mediated Mast Cell Activation","volume":"100","author":"Chen","year":"2000","journal-title":"Immunology"},{"key":"ref_279","doi-asserted-by":"crossref","first-page":"1070","DOI":"10.3109\/09553002.2010.501844","article-title":"Possible Role of Vitamin E, Coenzyme Q10 and Rutin in Protection against Cerebral Ischemia\/Reperfusion Injury in Irradiated Rats","volume":"86","author":"Rashed","year":"2010","journal-title":"Int. J. Radiat. Biol."},{"key":"ref_280","doi-asserted-by":"crossref","first-page":"629","DOI":"10.1016\/j.nutres.2008.06.004","article-title":"Protective Effect of Rutin against Spatial Memory Impairment Induced by Trimethyltin in Rats","volume":"28","author":"Koda","year":"2008","journal-title":"Nutr. Res."},{"key":"ref_281","doi-asserted-by":"crossref","first-page":"168","DOI":"10.1016\/j.ejphar.2006.04.004","article-title":"Central Nervous System Depressant Action of Flavonoid Glycosides","volume":"539","author":"Wasowski","year":"2006","journal-title":"Eur. J. Pharmacol."},{"key":"ref_282","doi-asserted-by":"crossref","first-page":"119252","DOI":"10.1016\/j.lfs.2021.119252","article-title":"The Bile Acid TUDCA and Neurodegenerative Disorders: An Overview","volume":"272","author":"Zangerolamo","year":"2021","journal-title":"Life Sci."},{"key":"ref_283","doi-asserted-by":"crossref","first-page":"4584","DOI":"10.1073\/pnas.0500815102","article-title":"Extremely Potent Triterpenoid Inducers of the Phase 2 Response: Correlations of Protection against Oxidant and Inflammatory Stress","volume":"102","author":"Liby","year":"2005","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_284","first-page":"336","article-title":"A Novel Synthetic Oleanane Triterpenoid, 2-Cyano-3,12-Dioxoolean-1,9-Dien-28-Oic Acid, with Potent Differentiating, Antiproliferative, and Anti-Inflammatory Activity1","volume":"59","author":"Suh","year":"1999","journal-title":"Cancer Res."},{"key":"ref_285","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1158\/1535-7163.MCT-06-0516","article-title":"Pharmacodynamic Characterization of Chemopreventive Triterpenoids as Exceptionally Potent Inducers of Nrf2-Regulated Genes","volume":"6","author":"Yates","year":"2007","journal-title":"Mol. Cancer Ther."},{"key":"ref_286","doi-asserted-by":"crossref","first-page":"1027","DOI":"10.1016\/S0960-894X(02)00105-1","article-title":"A Novel Dicyanotriterpenoid, 2-Cyano-3,12-Dioxooleana-1,9(11)-Dien-28-Onitrile, Active at Picomolar Concentrations for Inhibition of Nitric Oxide Production","volume":"12","author":"Honda","year":"2002","journal-title":"Bioorg. Med. Chem. Lett."},{"key":"ref_287","doi-asserted-by":"crossref","first-page":"3","DOI":"10.3233\/JHD-210510","article-title":"A Double-Pronged Sword: XJB-5-131 Is a Suppressor of Somatic Instability and Toxicity in Huntington\u2019s Disease","volume":"11","author":"Wipf","year":"2022","journal-title":"J. Huntingt. Dis."},{"key":"ref_288","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1111\/j.1527-3458.2007.00018.x","article-title":"Pharmacological Properties of BN82451: A Novel Multitargeting Neuroprotective Agent","volume":"13","author":"Chabrier","year":"2007","journal-title":"CNS Drug. Rev."},{"key":"ref_289","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1016\/0022-510X(92)90191-M","article-title":"Kynurenic Acid Concentrations Are Reduced in Huntington\u2019s Disease Cerebral Cortex","volume":"108","author":"Beal","year":"1992","journal-title":"J. Neurol. Sci."},{"key":"ref_290","doi-asserted-by":"crossref","first-page":"961","DOI":"10.1016\/j.cub.2011.04.028","article-title":"The Kynurenine Pathway Modulates Neurodegeneration in a Drosophila Model of Huntington\u2019s Disease","volume":"21","author":"Campesan","year":"2011","journal-title":"Curr. Biol."},{"key":"ref_291","doi-asserted-by":"crossref","first-page":"715","DOI":"10.1146\/annurev-biochem-061516-045037","article-title":"Oxidative Stress","volume":"86","author":"Sies","year":"2017","journal-title":"Annu. Rev. Biochem."},{"key":"ref_292","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1503\/jpn.100057","article-title":"N-Acetylcysteine in Psychiatry: Current Therapeutic Evidence and Potential Mechanisms of Action","volume":"36","author":"Dean","year":"2011","journal-title":"J. Psychiatry Neurosci."},{"key":"ref_293","doi-asserted-by":"crossref","first-page":"4117","DOI":"10.1016\/j.bbagen.2013.04.016","article-title":"The Chemistry and Biological Activities of N-Acetylcysteine","volume":"1830","author":"Samuni","year":"2013","journal-title":"Biochim. Biophys. Acta (BBA)\u2014Gen. Subj."}],"container-title":["Antioxidants"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2076-3921\/12\/3\/571\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T18:42:08Z","timestamp":1760121728000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2076-3921\/12\/3\/571"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,2,24]]},"references-count":293,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2023,3]]}},"alternative-id":["antiox12030571"],"URL":"https:\/\/doi.org\/10.3390\/antiox12030571","relation":{},"ISSN":["2076-3921"],"issn-type":[{"value":"2076-3921","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,2,24]]}}}