Journal article

Pioglitazone and Deoxyribonucleoside Combination Treatment Increases Mitochondrial Respiratory Capacity in m.3243A > G MELAS Cybrid Cells

Harrison J Burgin, M Isabel G Lopez Sanchez, Craig M Smith, Ian A Trounce, Matthew McKenzie

International Journal of Molecular Sciences | MDPI | Published : 2020

Abstract

The lack of effective treatments for mitochondrial disease has seen the development of new approaches, including those that aim to stimulate mitochondrial biogenesis to boost ATP generation above a critical disease threshold. Here, we examine the effects of the peroxisome proliferator-activated receptor γ (PPARγ) activator pioglitazone (PioG), in combination with deoxyribonucleosides (dNs), on mitochondrial biogenesis in cybrid cells containing >90% of the m.3243A>G mutation associated with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS). PioG + dNs combination treatment increased mtDNA copy number and mitochondrial mass in both control (CON) and m.3243A>G (MU..

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University of Melbourne Researchers

Grants

Awarded by National Health and Medical Research Grant


Funding Acknowledgements

This work was funded by Deakin University (H.J.B. and M.M.), an Australian Government Research Training Program Scholarship (H.J.B.), and the Australian Mito Foundation (H.J.B. and M.I.G.L.S.). I.T. was supported by National Health and Medical Research Grant GNT1159795. Centre for Eye Research Australia receives operational infrastructure support from the Victorian Government.