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Mitochondrial Neuropathology
Elucidating Neuronal Susceptibility to Mitochondrial Disease
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Featured publications

  • Home
    • Blog
    • Featured projects
    • Featured publications
    • Public Engagement
      • Community
      • Featured press appearances
  • QuintanaLab
  • SanzLab
  • Puig2Lab
  • CutandoLab
  • Naon, C., Castell, L., Thirard, S., Moreno, M., Rialle, S., Goetz, E., Casals, E., Rogliardo, A., Gut, M., Esteve-Codina, A., Quintana, A., Bertaso, F., Valjent, E., & Cutando, L. (2025). Translatome of dorsal striatum parvalbumin interneurons revisited: Insights across diverse experimental paradigms. Frontiers in Cellular Neuroscience, 19, 1648461.
  • Ulgen, D. H., Chioino, A., Zanoletti, O., Quintana, A., Sanz, E., & Sandi, C. (2025). Mitochondrial control of ciliary gene expression and structure in striatal neurons. The Journal of Physiology.
  • Sánchez-Benito L, González-Torres M, Fernández-González I, Cutando L, Royo M, Compte J, Vila M, Jurado S, Sanz E, & Quintana A. (2025). Dysfunctional LHX6 pallido-subthalamic projections mediate epileptic events in a mouse model of Leigh Syndrome. J Clin Invest.
  • Puighermanal E, Luna-Sánchez M, Gella A, van der Walt G, Urpi A, Royo M, Tena-Morraja P, Appiah I, de Donato MH, Menardy F, Bianchi P, Esteve-Codina A, Rodríguez-Pascau L, Vergara C, Gómez-Pallarès M, Marsicano G, Bellocchio L, Martinell M, Sanz E, Jurado S, Soriano FX, Pizcueta P, Quintana A. (2024). Cannabidiol ameliorates mitochondrial disease via PPARγ activation in preclinical models. Nature Communications, 15(1), 7730.
  • del Prado L, Jaraíz-Rodríguez M, Agro M, Zamora-Dorta M, Azpiazu N, Calleja M, Lopez-Manzaneda M, de Juan-Sanz J, Fernández-Rodrigo, A, Esteban JA, Girona, M., Quintana, A., & Balsa, E. (2024). Compensatory activity of the PC-ME1 metabolic axis underlies differential sensitivity to mitochondrial complex I inhibition. Nature Communications, 15(1), 8682.
  • Aguilar K, Canal C, Comes G, Díaz-Clavero S, Llanos MA, Quintana A, Sanz E, & Hidalgo J. (2024). Interleukin-6-elicited chronic neuroinflammation may decrease survival but is not sufficient to drive disease progression in a mouse model of Leigh syndrome. Journal of Inflammation, 21(1), 1.
  • Machuca-Márquez P, Sánchez-Benito L, Menardy F, Urpi A, Girona M, Puighermanal E, Appiah I, Palmiter RD, Sanz E, Quintana A. Vestibular CCK signaling drives motion sickness-like behavior in mice. Proceedings of the National Academy of Sciences. 2023;120(44):e2304933120.
  • Naón D, Hernández-Alvarez M I, Shinjo S, Wieczor M, Ivanova S, Martins de Brito O, Quintana A, Hidalgo J, Palacín M, Aparicio P, Castellanos J, Lores L, Sebastián D, Fernández-Veledo S, Vendrell J, Joven J, Orozco M, Zorzano A, & Scorrano L. (2023). Splice variants of mitofusin 2 shape the endoplasmic reticulum and tether it to mitochondria. Science, 380(6651), eadh9351.
  • Cutando L, Puighermanal E, Castell L, Tarot P, Belle M, Bertaso F, Arango-Lievano M, Ango F, Rubinstein M, Quintana A, Chédotal A, Mameli M, Valjent E. (2022).  Cerebellar dopamine D2 receptors regulate social behaviors. Nature Neuroscience. 25(7):900-911.
  • Kevin Aguilar K, Comes G, Canal C, Quintana A, Sanz E, Hidalgo J. (2022). Microglial response promotes neurodegeneration in the Ndufs4 KO mouse model of Leigh syndrome. GLIA. 70(11):2032-2044.
  • van de Wal M, Adjobo-Hermans M, Keijer J, Schirris T, Homberg J, Wieckowski MR, Grefte S, van Schothorst EM, van Karnebeek C, Quintana A, Werner JH Koopman. (2022) Ndufs4 knockout mouse models of Leigh syndrome: pathophysiology and intervention. Brain. 145(1):45-63.
  • Amo-Aparicio, J., Garcia-Garcia, J., Francos-Quijorna, I., Urpi, A., Esteve-Codina, A., Gut, M., Quintana, A., & Lopez-Vales, R. (2021). Interleukin-4 and interleukin-13 induce different metabolic profiles in microglia and macrophages that relate with divergent outcomes after spinal cord injury. Theranostics, 11(20), 9805–9820.
  • Luna-Sánchez M, Bianchi P, Quintana A. (2021) Mitochondria-Induced Immune Response as a Trigger for Neurodegeneration: A Pathogen from within. Int. J. Mol. Sci. 2021, 22(16), 8523.
  • Puighermanal E, Castell L, Esteve-Codina A, Melser S, Kaganovsky K, Zussy C, Boubaker-Vitre J, Gut M, Rialle S, Kellendonk C, Sanz E, Quintana A, Marsicano G, Martin M, Rubinstein M, Girault JA, Ding JB, Valjent E. (2020). Functional and Molecular Heterogeneity of D2R Neurons Along Dorsal Ventral Axis in the Striatum. Nat Commun. 23;11(1):1957.
  • Prada-Dacasa, P., Urpi, A., Sánchez-Benito, L., Bianchi, P., & Quintana, A. (2020). Measuring Breathing Patterns in Mice Using Whole-body Plethysmography. Bio-Protocol, 10(17), e3741.
  • Sharma, V., Sood, R., Khlaifia, A., Eslamizade, M. J., Hung, T.-Y., Lou, D., Asgarihafshejani, A., Lalzar, M., Kiniry, S. J., Stokes, M. P., Cohen, N., Nelson, A. J., Abell, K., Possemato, A. P., Gal-Ben-Ari, S., Truong, V. T., Wang, P., Yiannakas, A., Saffarzadeh, F., Cuello, A. C., Nader, K., Kaufman, R. J., Costa-Mattioli, M., Baranov, P. V., Quintana, A., Sanz, E., Khoutorsky, A., Lacaille, J.-C., Rosenblum, K., & Sonenberg, N. (2020). eIF2α controls memory consolidation via excitatory and somatostatin neurons. Nature, 586(7829), 412–416.
  • Miller, H. C., Louw, R., Mereis, M., Venter, G., Boshoff, J.-D., Mienie, L., van Reenen, M., Venter, M., Lindeque, J. Z., Domínguez-Martínez, A., Quintana, A., & van der Westhuizen, F. H. (2021). Metallothionein 1 Overexpression Does Not Protect Against Mitochondrial Disease Pathology in Ndufs4 Knockout Mice. Molecular Neurobiology, 58(1), 243–262.
  • Gella A, Prada-Dacasa P, Carrascal M, Urpi A, González-Torres M, Abian J, Sanz E, Quintana A. (2020). Mitochondrial Proteome of Affected Glutamatergic Neurons in a Mouse Model of Leigh Syndrome. Front Cell Dev Biol. 28;8:660.
  • Puighermanal, E., Castell, L., Esteve-Codina, A., Melser, S., Kaganovsky, K., Zussy, C., Boubaker-Vitre, J., Gut, M., Rialle, S., Kellendonk, C., Sanz, E., Quintana, A., Marsicano, G., Martin, M., Rubinstein, M., Girault, J.-A., Ding, J. B., & Valjent, E. (2020). Functional and molecular heterogeneity of D2R neurons along dorsal ventral axis in the striatum. Nature Communications, 11(1), 1957.
  • Bolea I, Gella A, Sanz E, Prada-Dacasa P, Menardy F, Bard AM, Machuca-Márquez P, Eraso-Pichot A, Mòdol-Caballero G, Navarro X, Kalume F, Quintana A. (2019). Defined neuronal populations drive fatal phenotype in a mouse model of Leigh syndrome. Elife. 8. pii: e47163.
  • Sanz E, Bean JC, Carey DP, Quintana A, McKnight GS. (2019). RiboTag: Ribosomal Tagging Strategy to Analyze Cell-Type-Specific mRNA Expression In Vivo. Curr  Protoc Neurosci. 88(1):e77.
  • Liu L, Zhang K, Sandoval H, Yamamoto S, Jaiswal M, Sanz E, Li Z, Hui J, Graham BH, Quintana A, Bellen HJ. (2015). Glial lipid droplets and ROS induced by mitochondrial defects promote neurodegeneration. Cell. 160(1-2):177-90.
  • Johnson SC, Yanos ME, Kayser EB, Quintana A, Sangesland M, Castanza A, Uhde L, Hui J, Wall VZ, Gagnidze A, Oh K, Wasko BM, Ramos FJ, Palmiter RD, Rabinovitch PS, Morgan PG, Sedensky MM, Kaeberlein M. (2013). mTOR inhibition alleviates mitochondrial disease in a mouse model of Leigh syndrome. Science. 342(6165):1524-8. Featured in Science Express in Science and Nature Medicine

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