Cannabidiol ameliorates mitochondrial disease via PPARγ activation in preclinical models
| dc.rights.license | open | en_US |
| dc.contributor.author | PUIGHERMANAL, Emma | |
| dc.contributor.author | LUNA-SÁNCHEZ, Marta | |
| dc.contributor.author | GELLA, Alejandro | |
| dc.contributor.author | VAN DER WALT, Gunter | |
| dc.contributor.author | URPI, Andrea | |
| dc.contributor.author | ROYO, María | |
| dc.contributor.author | TENA-MORRAJA, Paula | |
| dc.contributor.author | APPIAH, Isabella | |
| dc.contributor.author | DE DONATO, Maria Helena | |
| dc.contributor.author | MENARDY, Fabien | |
| dc.contributor.author | BIANCHI, Patrizia | |
| dc.contributor.author | ESTEVE-CODINA, Anna | |
| dc.contributor.author | RODRÍGUEZ-PASCAU, Laura | |
| dc.contributor.author | VERGARA, Cristina | |
| dc.contributor.author | GÓMEZ-PALLARÈS, Mercè | |
| hal.structure.identifier | Neurocentre Magendie : Physiopathologie de la Plasticité Neuronale [U1215 Inserm - UB] | |
| dc.contributor.author | MARSICANO, Giovanni | |
| hal.structure.identifier | Neurocentre Magendie : Physiopathologie de la Plasticité Neuronale [U1215 Inserm - UB] | |
| dc.contributor.author | BELLOCCHIO, Luigi | |
| dc.contributor.author | MARTINELL, Marc | |
| dc.contributor.author | SANZ, Elisenda | |
| dc.contributor.author | JURADO, Sandra | |
| dc.contributor.author | SORIANO, Francesc Xavier | |
| dc.contributor.author | PIZCUETA, Pilar | |
| dc.contributor.author | QUINTANA, Albert | |
| dc.date.accessioned | 2025-05-19T14:01:16Z | |
| dc.date.available | 2025-05-19T14:01:16Z | |
| dc.date.issued | 2024-09-04 | |
| dc.identifier.issn | 2041-1723 | en_US |
| dc.identifier.uri | https://oskar-bordeaux.fr/handle/20.500.12278/206656 | |
| dc.description.abstractEn | Mutations in mitochondrial energy-producing genes lead to a heterogeneous group of untreatable disorders known as primary mitochondrial diseases (MD). Leigh syndrome (LS) is the most common pediatric MD and is characterized by progressive neuromuscular affectation and premature death. Here, we show that daily cannabidiol (CBD) administration significantly extends lifespan and ameliorates pathology in two LS mouse models, and improves cellular function in fibroblasts from LS patients. CBD delays motor decline and neurodegenerative signs, improves social deficits and breathing abnormalities, decreases thermally induced seizures, and improves neuropathology in affected brain regions. Mechanistically, we identify peroxisome proliferator-activated receptor gamma (PPARγ) as a key nuclear receptor mediating CBD’s beneficial effects, while also providing proof of dysregulated PPARγ expression and activity as a common feature in both mouse neurons and fibroblasts from LS patients. Taken together, our results provide the first evidence for CBD as a potential treatment for LS. © The Author(s) 2024. | |
| dc.language.iso | EN | en_US |
| dc.rights | Attribution-NonCommercial-NoDerivs 3.0 United States | * |
| dc.rights.uri | http://creativecommons.org/licenses/by-nc-nd/3.0/us/ | * |
| dc.title.en | Cannabidiol ameliorates mitochondrial disease via PPARγ activation in preclinical models | |
| dc.title.alternative | Nat Commun | en_US |
| dc.type | Article de revue | en_US |
| dc.identifier.doi | 10.1038/s41467-024-51884-8 | en_US |
| dc.subject.hal | Sciences du Vivant [q-bio]/Neurosciences [q-bio.NC] | en_US |
| dc.identifier.pubmed | 39231983 | en_US |
| dc.description.sponsorshipEurope | Elucidating Neuronal Susceptibility to Mitochondrial Disease | en_US |
| bordeaux.journal | Nature Communications | en_US |
| bordeaux.volume | 15 | en_US |
| bordeaux.hal.laboratories | Neurocentre Magendie - U1215 | en_US |
| bordeaux.issue | 1 | en_US |
| bordeaux.institution | Université de Bordeaux | en_US |
| bordeaux.institution | INSERM | en_US |
| bordeaux.team | Endocannabinoïdes et Neuroadaptation | en_US |
| bordeaux.peerReviewed | oui | en_US |
| bordeaux.inpress | non | en_US |
| hal.identifier | hal-05073865 | |
| hal.version | 1 | |
| hal.date.transferred | 2025-05-19T14:01:22Z | |
| hal.popular | non | en_US |
| hal.audience | Internationale | en_US |
| hal.export | true | |
| dc.rights.cc | CC BY-NC-ND | en_US |
| bordeaux.COinS | ctx_ver=Z39.88-2004&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.jtitle=Nature%20Communications&rft.date=2024-09-04&rft.volume=15&rft.issue=1&rft.eissn=2041-1723&rft.issn=2041-1723&rft.au=PUIGHERMANAL,%20Emma&LUNA-S%C3%81NCHEZ,%20Marta&GELLA,%20Alejandro&VAN%20DER%20WALT,%20Gunter&URPI,%20Andrea&rft.genre=article |