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2024-09-04

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cc-by (c) Puighermanal E et al., 2024
Si us plau utilitzeu sempre aquest identificador per citar o enllaçar aquest document: https://hdl.handle.net/2445/217187

Cannabidiol ameliorates mitochondrial disease via PPARγ activation in preclinical models

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https://doi.org/10.1038/s41467-024-51884-8

Resum

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.

Matèries

Malalties neuromusculars, Cànnabis, ADN mitocondrial

Matèries (anglès)

Neuromuscular diseases, Cannabis, Mitochondrial DNA

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Articles publicats en revistes (Biologia Cel·lular, Fisiologia i Immunologia)

Citació

PUIGHERMANAL, Emma, LUNA-SÁNCHEZ, Marta, GELLA, Alejandro, VAN DER WALT, Gunter, URPI, Andrea, ROYO, María, TENA-MORRAJA, Paula, APPIAH, Isabella, DE DONATO, Maria helena, MENARDY, Fabien, BIANCHI, Patrizia, ESTEVE-CODINA, Anna, RODRÍGUEZ-PASCAU, Laura, VERGARA PAÑOS, Cristina, GÓMEZ-PALLARÈS, Mercè, MARSICANO, Giovanni, BELLOCCHIO, Luigi, MARTINELL, Marc, SANZ, Elisenda, JURADO, Sandra, SORIANO ZARAGOZA, Francesc x. (francesc xavier), PIZCUETA LALANZA, María pilar, QUINTANA, Albert. Cannabidiol ameliorates mitochondrial disease via PPARγ activation in preclinical models. _Nature Communications_. 2024. Vol. 15, núm. 1, pàgs. 1-21. [consulta: 28 de gener de 2026]. ISSN: 2041-1723. [Disponible a: https://hdl.handle.net/2445/217187]

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