We found that the pharmacological stimulation of Peroxisome Proliferator-Activated Receptor Gamma (PPARG) by Pioglitazone upregulates IDE and also PITRM1 protein levels restoring the presequence processing machinery and improving Frataxin maturation and mitochondrial function.
Our findings provide mechanistic insights and suggest a potential pharmacological strategy for this rare neurodegenerative mitochondrial disease.
Tuesday, July 11, 2023
PPAR-gamma agonist pioglitazone recovers mitochondrial quality controls in fibroblasts from PITRM1-deficient patients
DArio Brunetti, A. D., Donfrancesco, C., Berlingieri, C., Frascarelli, M., Giacomello, A. P., Magalhaes Rebelo, L., Bindoff, S., Reeval, P., Filippo, M., Santorelli, G., Massaro, C. F., Viscomi, M., & Zeviani, D. (s/f). PPAR-gamma agonist pioglitazone recovers mitochondrial quality controls in fibroblasts from PITRM1-deficient patients. Front. Pharmacol. Sec. Experimental Pharmacology and Drug Discovery, 14. doi:10.3389/fphar.2023.1220620
