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In the present study, we characterized the changes in mitochondrial function and biogenesis markers in two MJD models, CMVMJD135 (MJD135) transgenic mice at a fully established phenotype stage and tetracycline-regulated PC6-3 Q108 cell line expressing mutant ataxin-3 (mATXN3). We detected mATXN3 in the mitochondrial fractions of PC6-3 Q108 cells, suggesting the interaction of expanded ATXN3 with the organelle. Interestingly, in both the cerebella of the MJD135 mouse model and in PC6-3 Q108 cells, we found decreased mitochondrial respiration, ATP production and mitochondrial membrane potential, strongly suggesting mitochondrial dysfunction in MJD. Also, in PC6-3 Q108 cells, an additional enhanced glycolytic flux was observed. Supporting the functional deficits observed in MJD mitochondria, MJD135 mouse cerebellum and PC6-3 Q108 cells showed reduced cytochrome c mRNA and protein levels. Overall, our findings show compromised mitochondrial function associated with decreased cytochrome c levels in both cell and animal models of MJD.<\/jats:p>","DOI":"10.3390\/cells12192397","type":"journal-article","created":{"date-parts":[[2023,10,3]],"date-time":"2023-10-03T07:28:29Z","timestamp":1696318109000},"page":"2397","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Mitochondrial Dysfunction and Decreased Cytochrome c in Cell and Animal Models of Machado\u2013Joseph Disease"],"prefix":"10.3390","volume":"12","author":[{"given":"Filipa","family":"Almeida","sequence":"first","affiliation":[{"name":"CNC-UC-Center for Neuroscience and Cell Biology, University of Coimbra, 3004-504 Coimbra, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ildete L.","family":"Ferreira","sequence":"additional","affiliation":[{"name":"CNC-UC-Center for Neuroscience and Cell Biology, University of Coimbra, 3004-504 Coimbra, Portugal"},{"name":"IIIUC-Institute for Interdisciplinary Research, University of Coimbra, 3030-789 Coimbra, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0886-4634","authenticated-orcid":false,"given":"Luana","family":"Naia","sequence":"additional","affiliation":[{"name":"CNC-UC-Center for Neuroscience and Cell Biology, University of Coimbra, 3004-504 Coimbra, Portugal"},{"name":"IIIUC-Institute for Interdisciplinary Research, University of Coimbra, 3030-789 Coimbra, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniela","family":"Marinho","sequence":"additional","affiliation":[{"name":"CNC-UC-Center for Neuroscience and Cell Biology, University of Coimbra, 3004-504 Coimbra, Portugal"},{"name":"IIIUC-Institute for Interdisciplinary Research, University of Coimbra, 3030-789 Coimbra, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana Catarina","family":"Vila\u00e7a-Ferreira","sequence":"additional","affiliation":[{"name":"Life and Health Sciences Research Institute (ICVS), School of Medicine, University of Minho, 4710-057 Braga, Portugal"},{"name":"ICVS\/3B\u2019s-PT Government Associate Laboratory, 4805-017 Guimar\u00e3es, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6247-0548","authenticated-orcid":false,"given":"Marta D.","family":"Costa","sequence":"additional","affiliation":[{"name":"Life and Health Sciences Research Institute (ICVS), School of Medicine, University of Minho, 4710-057 Braga, Portugal"},{"name":"ICVS\/3B\u2019s-PT Government Associate Laboratory, 4805-017 Guimar\u00e3es, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9680-4027","authenticated-orcid":false,"given":"Sara","family":"Duarte-Silva","sequence":"additional","affiliation":[{"name":"Life and Health Sciences Research Institute (ICVS), School of Medicine, University of Minho, 4710-057 Braga, Portugal"},{"name":"ICVS\/3B\u2019s-PT Government Associate Laboratory, 4805-017 Guimar\u00e3es, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0920-6350","authenticated-orcid":false,"given":"Patr\u00edcia","family":"Maciel","sequence":"additional","affiliation":[{"name":"Life and Health Sciences Research Institute (ICVS), School of Medicine, University of Minho, 4710-057 Braga, Portugal"},{"name":"ICVS\/3B\u2019s-PT Government Associate Laboratory, 4805-017 Guimar\u00e3es, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0700-3776","authenticated-orcid":false,"given":"A. Cristina","family":"Rego","sequence":"additional","affiliation":[{"name":"CNC-UC-Center for Neuroscience and Cell Biology, University of Coimbra, 3004-504 Coimbra, Portugal"},{"name":"FMUC-Faculty of Medicine, University of Coimbra, 3000-548 Coimbra, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,10,3]]},"reference":[{"key":"ref_1","first-page":"139","article-title":"Epidemiology and clinical aspects of Machado-Joseph disease","volume":"61","author":"Sequeiros","year":"1993","journal-title":"Adv. Neurol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"174","DOI":"10.1159\/000358801","article-title":"The Global Epidemiology of Hereditary Ataxia and Spastic Paraplegia: A Systematic Review of Prevalence Studies","volume":"42","author":"Ruano","year":"2014","journal-title":"Neuroepidemiology"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"920","DOI":"10.1007\/s10038-008-0330-y","article-title":"Analysis of segregation patterns in Machado\u2013Joseph disease pedigrees","volume":"53","author":"Bettencourt","year":"2008","journal-title":"J. Hum. Genet."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1111\/jnc.14541","article-title":"Machado\u2013Joseph disease\/spinocerebellar ataxia type 3: Lessons from disease pathogenesis and clues into therapy","volume":"148","author":"Matos","year":"2019","journal-title":"J. Neurochem."},{"key":"ref_5","doi-asserted-by":"crossref","unstructured":"Chen, Y.-S., Hong, Z.-X., Lin, S.-Z., and Harn, H.-J. (2020). Identifying Therapeutic Targets for Spinocerebellar Ataxia Type 3\/Machado\u2013Joseph Disease through Integration of Pathological Biomarkers and Therapeutic Strategies. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21093063"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1069","DOI":"10.1016\/j.bbrc.2016.07.122","article-title":"Mitochondrial Ca2+ handling in Huntington\u2019s and Alzheimer\u2019s diseases-Role of ER-mitochondria crosstalk","volume":"483","author":"Naia","year":"2017","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1016\/j.bbadis.2011.10.010","article-title":"Compromised mitochondrial complex II in models of Machado\u2013Joseph disease","volume":"1822","author":"Oliveira","year":"2012","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"368","DOI":"10.3389\/fnmol.2018.00368","article-title":"Mitochondrial Morphology, Function and Homeostasis Are Impaired by Expression of an N-terminal Calpain Cleavage Fragment of Ataxin-3","volume":"11","author":"Harmuth","year":"2018","journal-title":"Front. Mol. Neurosci."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1430","DOI":"10.1007\/s11064-017-2199-7","article-title":"Expanded and Wild-type Ataxin-3 Modify the Redox Status of SH-SY5Y Cells Overexpressing \u03b1-Synuclein","volume":"42","author":"Noronha","year":"2017","journal-title":"Neurochem. Res."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1884","DOI":"10.1002\/jnr.22011","article-title":"Decreased antioxidant enzyme activity and increased mitochondrial DNA damage in cellular models of Machado-Joseph disease","volume":"87","author":"Yu","year":"2009","journal-title":"J. Neurosci. Res."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"206","DOI":"10.1159\/000339207","article-title":"Patterns of Mitochondrial DNA Damage in Blood and Brain Tissues of a Transgenic Mouse Model of Machado-Joseph Disease","volume":"11","author":"Kazachkova","year":"2013","journal-title":"Neurodegener. Dis."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"449","DOI":"10.1007\/s12031-014-0360-1","article-title":"Differential mtDNA Damage Patterns in a Transgenic Mouse Model of Machado\u2013Joseph Disease (MJD\/SCA3)","volume":"55","author":"Ramos","year":"2015","journal-title":"J. Mol. Neurosci."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1016\/j.neuint.2017.10.013","article-title":"Mass spectrometry analyses of normal and polyglutamine expanded ataxin-3 reveal novel interaction partners involved in mitochondrial function","volume":"112","author":"Kristensen","year":"2018","journal-title":"Neurochem. Int."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"30436","DOI":"10.1038\/srep30436","article-title":"Far-infrared radiation protects viability in a cell model of Spinocerebellar Ataxia by preventing polyQ protein accumulation and improving mitochondrial function","volume":"6","author":"Chang","year":"2016","journal-title":"Sci. Rep."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"815","DOI":"10.1002\/mds.27292","article-title":"Neuroprotective Effects of Creatine in the CMVMJD135 Mouse Model of Spinocerebellar Ataxia Type 3","volume":"33","author":"Silva","year":"2018","journal-title":"Mov. Disord."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"658339","DOI":"10.3389\/fnmol.2021.658339","article-title":"Broad Influence of Mutant Ataxin-3 on the Proteome of the Adult Brain, Young Neurons, and Axons Reveals Central Molecular Processes and Biomarkers in SCA3\/MJD Using Knock-In Mouse Model","volume":"14","author":"Wiatr","year":"2021","journal-title":"Front. Mol. Neurosci."},{"key":"ref_17","doi-asserted-by":"crossref","unstructured":"Harmuth, T., Weber, J.J., Zimmer, A.J., Sowa, A.S., Schmidt, J., Fitzgerald, J.C., Sch\u00f6ls, L., Riess, O., and H\u00fcbener-Schmid, J. (2022). Mitochondrial Dysfunction in Spinocerebellar Ataxia Type 3 Is Linked to VDAC1 Deubiquitination. Int. J. Mol. Sci., 23.","DOI":"10.3390\/ijms23115933"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"3669","DOI":"10.1523\/JNEUROSCI.13-09-03669.1993","article-title":"A system for characterizing cellular and molecular events in programmed neuronal cell death","volume":"13","author":"Pittman","year":"1993","journal-title":"J. Neurosci."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1007\/s13311-013-0255-9","article-title":"Chronic Treatment with 17-DMAG Improves Balance and Coordination in A New Mouse Model of Machado-Joseph Disease","volume":"11","author":"Amorim","year":"2014","journal-title":"Neurotherapeutics"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1016\/j.nbd.2010.05.021","article-title":"Motor uncoordination and neuropathology in a transgenic mouse model of Machado\u2013Joseph disease lacking intranuclear inclusions and ataxin-3 cleavage products","volume":"40","author":"Costa","year":"2010","journal-title":"Neurobiol. Dis."},{"key":"ref_21","doi-asserted-by":"crossref","unstructured":"Precious, S.V., Rosser, A.E., and Dunnett, S.B. (2018). Huntington\u2019s Disease, Springer.","DOI":"10.1007\/978-1-4939-7825-0"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1016\/B978-0-12-416618-9.00005-4","article-title":"Techniques to Monitor Glycolysis","volume":"542","author":"TeSlaa","year":"2014","journal-title":"Methods Enzymol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1016\/0003-2697(85)90405-1","article-title":"Simultaneous extraction and reverse-phase high-performance liquid chromatographic determination of adenine and pyridine nucleotides in human red blood cells","volume":"146","author":"Stocchi","year":"1985","journal-title":"Anal. Biochem."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"652","DOI":"10.1111\/jnc.13165","article-title":"Ca2+ handling in isolated brain mitochondria and cultured neurons derived from the YAC128 mouse model of Huntington\u2019s disease","volume":"134","author":"Pellman","year":"2015","journal-title":"J. Neurochem."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1016\/j.nbd.2016.06.012","article-title":"Early-onset Purkinje cell dysfunction underlies cerebellar ataxia in peroxisomal multifunctional protein-2 deficiency","volume":"94","author":"Verheijden","year":"2016","journal-title":"Neurobiol. Dis."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"676","DOI":"10.1038\/nmeth.2019","article-title":"Fiji: An open-source platform for biological-image analysis","volume":"9","author":"Schindelin","year":"2012","journal-title":"Nat. Methods"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/S0003-9861(02)00585-4","article-title":"In vitro effects of polyglutamine tracts on Ca2+-dependent depolarization of rat and human mitochondria: Relevance to Huntington\u2019s disease","volume":"410","author":"Panov","year":"2003","journal-title":"Arch. Biochem. Biophys."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1042\/bse0470053","article-title":"Mitochondrial proton and electron leaks","volume":"47","author":"Jastroch","year":"2010","journal-title":"Essays Biochem."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"780","DOI":"10.1097\/01.WCB.0000122743.72175.52","article-title":"Quantitative RT-PCR Analysis of Uncoupling Protein Isoforms in Mouse Brain Cortex: Methodological Optimization and Comparison of Expression with Brown Adipose Tissue and Skeletal Muscle","volume":"24","author":"Lengacher","year":"2004","journal-title":"J. Cereb. Blood Flow Metab."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1006\/nbdi.1998.0208","article-title":"Heterogeneous intracellular localization and expression of ataxin-3","volume":"5","author":"Trottier","year":"1998","journal-title":"Neurobiol. Dis."},{"key":"ref_31","first-page":"363","article-title":"Control mechanisms in mitochondrial oxidative phosphorylation","volume":"8","year":"2013","journal-title":"Neural. Regen. Res."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1042\/bse0470001","article-title":"The mitochondrial respiratory chain","volume":"47","author":"Rich","year":"2010","journal-title":"Essays Biochem."},{"key":"ref_33","doi-asserted-by":"crossref","unstructured":"Lopes, C., Ferreira, I.L., Maranga, C., Beatriz, M., Mota, S.I., Sereno, J., Castelhano, J., Abrunhosa, A., Oliveira, F., and De Rosa, M. (2022). Mitochondrial and redox modifications in early stages of Huntington\u2019s disease. Redox Biol., 56.","DOI":"10.1016\/j.redox.2022.102424"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"4963","DOI":"10.1242\/jcs.113662","article-title":"PGC1\u03b1 and mitochondrial metabolism\u2013emerging concepts and relevance in ageing and neurodegenerative disorders","volume":"125","author":"Austin","year":"2012","journal-title":"J. Cell Sci."},{"key":"ref_35","first-page":"1153","article-title":"Transgenic expression and activation of PGC-1\u03b1 protect dopaminergic neurons in the MPTP mouse model of Parkinson\u2019s disease","volume":"69","author":"Tselykh","year":"2011","journal-title":"Cell. Mol. Life Sci."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"626","DOI":"10.1111\/ejn.13157","article-title":"Peroxisome proliferator-activated receptor-\u03b3 coactivator-1\u03b1 mediates neuroprotection against excitotoxic brain injury in transgenic mice: Role of mitochondria and X-linked inhibitor of apoptosis protein","volume":"43","author":"Pham","year":"2016","journal-title":"Eur. J. Neurosci."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1016\/j.cell.2006.09.024","article-title":"Suppression of Reactive Oxygen Species and Neurodegeneration by the PGC-1 Transcriptional Coactivators","volume":"127","author":"Drori","year":"2006","journal-title":"Cell"},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"3225","DOI":"10.1091\/mbc.e07-05-0404","article-title":"The Mitochondrial Transcription Factor TFAM Coordinates the Assembly of Multiple DNA Molecules into Nucleoid-like Structures","volume":"18","author":"Kaufman","year":"2007","journal-title":"Mol. Biol. Cell"},{"key":"ref_39","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_40","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_41","doi-asserted-by":"crossref","first-page":"166","DOI":"10.1016\/S0166-2236(99)01534-9","article-title":"Mitochondrial membrane potential and neuronal glutamate excitotoxicity: Mortality and millivolts","volume":"23","author":"Nicholls","year":"2000","journal-title":"Trends Neurosci."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"98","DOI":"10.2144\/000113610","article-title":"Mitochondrial membrane potential probes and the proton gradient: A practical usage guide","volume":"50","author":"Perry","year":"2011","journal-title":"BioTechniques"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"182","DOI":"10.1016\/j.expneurol.2006.03.029","article-title":"Potassium channel dysfunction and depolarized resting membrane potential in a cell model of SCA3","volume":"201","author":"Jeub","year":"2006","journal-title":"Exp. Neurol."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"12713","DOI":"10.1523\/JNEUROSCI.3909-08.2008","article-title":"Deranged Calcium Signaling and Neurodegeneration in Spinocerebellar Ataxia Type 3","volume":"28","author":"Chen","year":"2008","journal-title":"J. Neurosci."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1016\/j.neuint.2009.11.003","article-title":"Polyglutamine-expanded ataxin-7 causes cerebellar dysfunction by inducing transcriptional dysregulation","volume":"56","author":"Chou","year":"2010","journal-title":"Neurochem. Int."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"196","DOI":"10.3389\/fnmol.2017.00196","article-title":"The Truncated C-terminal Fragment of Mutant ATXN3 Disrupts Mitochondria Dynamics in Spinocerebellar Ataxia Type 3 Models","volume":"10","author":"Hsu","year":"2017","journal-title":"Front. Mol. Neurosci."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"1017","DOI":"10.1007\/s13311-021-01022-9","article-title":"The Sigma-1 Receptor Mediates Pridopidine Rescue of Mitochondrial Function in Huntington Disease Models","volume":"18","author":"Naia","year":"2021","journal-title":"Neurotherapeutics"}],"container-title":["Cells"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2073-4409\/12\/19\/2397\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T21:04:27Z","timestamp":1760130267000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2073-4409\/12\/19\/2397"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,10,3]]},"references-count":47,"journal-issue":{"issue":"19","published-online":{"date-parts":[[2023,10]]}},"alternative-id":["cells12192397"],"URL":"https:\/\/doi.org\/10.3390\/cells12192397","relation":{},"ISSN":["2073-4409"],"issn-type":[{"value":"2073-4409","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,10,3]]}}}