Poster preview
ePoster
MULTI-MODAL PROFILING UNCOVERS CHOROID PLEXUS–DRIVEN IMMUNOMETABOLIC CHANGES IN LEIGH SYNDROME AND THEIR MODULATION BY CANNABIDIOL
Lotte de Ridderand 7 co-authors
Neuroscience Institute, Department of Cell Biology, Physiology and Immunology, Autonomous University of Barcelona
FENS Forum 2026 (2026)
Barcelona, Spain
Board PS06-09PM-073
Presentation
Date TBA
Board: PS06-09PM-073
Event Information
Poster Board
PS06-09PM-073
Poster
View posterAbstract
Leigh syndrome (LS) is a devastating primary mitochondrial disease characterized by neuroinflammation, oxidative stress, lactic acidosis, and severe neurological impairments, with no approved treatments to date. Emerging evidence highlights a dysregulated immune response as a critical driver of LS pathology, with the choroid plexus (ChP) -a central neuroimmunometabolic hub- emerging as a key but understudied player. In this study, we used LS mouse models lacking the mitochondrial respiratory chain protein NDUFS4 to investigate mitochondrial dysfunction in high-energy-demanding cells, such as neurons and ChP cells. RNAseq, immunoblot, and immunofluorescence analyses revealed activation of type I IFN/antiviral and innate immune responses in the ChP, as well as in the neighboring brain parenchyma. Moreover, we found that ChP dysregulation alters the production and release of metabolic factors, such as lactate, which may contribute to a feedback loop that exacerbates neuroinflammation and immunometabolic imbalance, thereby driving disease progression. Notably, multi-omic analyses showed that chronic cannabidiol (CBD) treatment, a cannabinoid agent previously reported to increase lifespan and ameliorate pathology in LS mouse models, dampens exacerbated innate immune/antiviral responses and normalizes specific ChP markers. These findings provide novel insight into LS neuropathology and identify potential treatments and biomarkers to address this clear unmet need.
Recommended posters
CO-TARGETING THE ENDOCANNABINOID AND MELATONINERGIC SYSTEMS MODULATES LIPID DYNAMICS IN MICROGLIA
Giovanna Vitolo, Mariarosaria Cammarota, Annalida Bedini, Maria Domercq, Silvia Rivara, Francesca Boscia
ASTROCYTIC MITOPHAGY PROTECTS AGAINST PROTEIN AGGREGATION-MEDIATED BIOENERGETIC STRESS AND MOTOR DISCOORDINATION
Darwin Israel Manjarrés Raza, Daniel Jimenez-Blasco, Nadia Di Franco, Veronica Bobo-Jimenez, Alba Pereda-Velarde, Dario Garcia-Rodriguez1, Silvia Gomila, Cristina Rodriguez, Carlos Costas-Insua2, Silvia Ginés, Manuel Guzman, Angeles Almeida, Ruben Quintana-Cabrera, Juan P. Bolaños
TARGETING LIPID METABOLISM PRESERVES MOTOR NEURONS AND RESTORES GLUCOSE UTILIZATION IN ALS
Viktória Reháková, Yuanyuan Zeng, Amanda Krøger, Anne Skøttrup Mørkholt, Caroline C. Real, Carsten Scavenius, Claire F. Meehan, Anne Landau, John Nieland, Steinunn Sara Helgudottir
NEURON-SPECIFIC METABOLIC DYSFUNCTION TRIGGERS SYSTEMIC REMODELING IN A PARKINSONIAN MOUSE MODEL
Claudia García-Rodríguez, Alejandro Jiménez-Medina, Esther García-Benitez, José López-Barneo, Xavier d’Anglemont de Tassigny, Patricia González-Rodríguez
CANNABIDIOL REDUCES NEUROINFLAMMATION CORRELATED WITH CB2 RECEPTOR CHANGES IN AN APP MOUSE MODEL OF ALZHEIMER’S DISEASE.
Shuo Wu, Andi Chan, Weicong Zhang, Jialin Li, Shahad Alotaibi, Afia Ali
CANNABIDIOL IN ALZHEIMER’S DISEASE: A MULTI-TARGET APPROACH TO PATHOLOGY AND COGNITIVE DYSFUNCTION
Jaume Lillo Jové, Iu Raïch, Rebassa Joan Biel, Martí Xutglà, Griñán Christian, Aina Bellver-Sanchis, Irene Reyes-Resina, Rafael Franco, Mercè Pallàs, Gemma Navarro