Organisation profile
Organisation profile
The Mitochondrial Neuropathology SGR group is devoted to elucidating the mechanisms whereby mitochondrial dysfunction leads to neurodegeneration to develop novel treatments for pathologies with mitochondrial involvement, including rare diseases caused by mutations of mitochondrial genes, the so-called primary mitochondrial disease (MD), as well as neurodegenerative diseases such as Alzheimer's disease (AD), which very particularly affect mitochondria, necessary for providing most of the energy to cells. Thus, the overarching goal of this SGR is the implementation of Mitochondrial Medicine, defined as a basic and clinical discipline dedicated to understanding, diagnosing and treating MD and mitochondrion-involved disease such as AD. To that end, we focus on 3 axes; 1) characterizing cellular and neuralcircuit heterogeneity and disease-specificity, 2) developing new tools for manipulation and in vivo exploration of mitochondrial activity, and 3) clinical translation.
Directed by:
Albert Quintana Romero
Research lines
- Research lines:
- Elucidating neuronal susceptibility to mitochondrial disease
- Identification of novel cannabionoid-based therapies for mitochondrial disease
- Development of novel tools to unlock cell type-specific neuronal function
- Neurobiology of innate behaviors
- Development of novel diagnostic kits for antibiotic resistances
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Collaborations and top research areas from the last five years
Profiles
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Laura Cutando Ruiz
- Department of Cellular Biology, Physiology and Immunology
- Institute of Neuroscience (INc)
- Mitochondrial Neuropathology
Person: Ramón Y Cajal researcher's programme, Affiliation to research
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TRANSCANN: Projecte: Decoding Cell Type-Specific Translatomics in Memory and Structural Plasticity Changes Induced by Cannabis
Puighermanal Puigvert, E. (Principal Investigator)
1/04/25 → 31/03/27
Project: Research Projects and Other Grants
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DEFINING THE ROLE OF KISS1 NEURONS IN THE ROSTRAL PERIVENTRICULAR AREA OF THE THIRD VENTRICLE (RP3V) IN MALE REPRODUCTIVE HEALTH
Sanz Iglesias, E. (Principal Investigator), Appiah , I. (Collaborator), Blanco Ramos, M. (Collaborator), Quintana Romero, A. (Collaborator), Urpi Badell, A. (Collaborator), Canal Capdevila, C. (Collaborator), Fernandez Gonzalez, I. (Collaborator) & Girona del Pozo, M. (Collaborator)
1/09/23 → 31/08/26
Project: Research Projects and Other Grants
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PrevGut: Preventing Toxic Protein Aggregates in the Gut
Sánchez Groot, N. (Principal Investigator), Fernandez Gallegos, M. R. (Investigator), Dominguez Martinez, A. (Others), PORCAR MIRALLES, M. (Others), Ramos Illan, S. (Others), Seira Curto, J. (Predoctoral) & Pérez Collell, G. (Technician)
2/12/24 → 1/06/25
Project: Research Projects and Other Grants
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Col6a1 knock-in mice provide a promising pre-clinical model for collagen VI-related dystrophies
López-Márquez, A., Badosa, C., Enjuanes-Ruiz, L., Hernández-Carabias, P., Sánchez-Martín, M., Cadot, B., Guesmia, Z., Georvasilis, I., Balsells, S., Blanco-Ramos, M., Puighermanal, E., Quintana, A., Roldán, M., Allamand, V. & Jiménez-Mallebrera, C., Jan 2026, In: Disease models & mechanisms. 19, 1, 12 p., dmm052460.Research output: Contribution to journal › Article › Research › peer-review
Open Access1 Link opens in a new tab Citation (Scopus) -
Dysfunctional LHX6 pallido-subthalamic projections mediate epileptic events in a mouse model of Leigh syndrome
González Torres, M., Fernández González, I., Cutando Ruiz, L., Royo, M., Compte, J., Vila Bover, M., Jurado, S., Sanz, E. & Quintana Romero, A., 2025, In: Journal of Clinical Investigation.Research output: Contribution to journal › Article › Research › peer-review
Open Access -
Exogenous prion-like proteins and their potential to trigger cognitive dysfunction
Curto, J. S., Martinez, A. D., Collell, G. P., Simon, E. B., Ruiz, M. R., Bordés, B. F., Sotillo, P. S., Hernandez, S. V., Fernandez, M. R. & de Groot, N. S., 27 May 2025, In: Molecular Systems Biology. 21, 8, p. 1-26 26 p., 12134.Research output: Contribution to journal › Article › Research › peer-review
Open Access1 Link opens in a new tab Citation (Scopus)
Student theses
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Estudio de la implicación del globo pálido en la epilepsia inducida por disfunción mitocondrial
Sanchez Benito, L. (Author), Sanz Iglesias, E. (Director) & Quintana Romero, A. (Director), 18 Oct 2023Student thesis: Doctoral thesis
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Investigation into the protection of mitochondrial disease pathology by metallothionein overexpression
Miller, H. (Author), Quintana Romero, A. (Director) & van der Westhuizen, F. H. (Director), Jul 2020Student thesis: Doctoral thesis
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Estudio del perfil traduccional citosólico y mitocondrial de poblaciones neuronales susceptibles a la deficiencia del complejo I mitocondrial
Prada Dacasa, P. (Author), Quintana Romero, A. (Director), Sanz Iglesias, E. (Director) & Hidalgo Pareja, J. (Tutor), 11 Dec 2022Student thesis: Doctoral thesis