Journal article
Study of mitochondrial respiratory defects on reprogramming to human induced pluripotent stem cells
SSC Hung, NJ Van Bergen, S Jackson, H Liang, DA Mackey, D Hernández, SY Lim, AW Hewitt, I Trounce, A Pébay, RCB Wong
Aging | IMPACT JOURNALS LLC | Published : 2016
Abstract
Reprogramming of somatic cells into a pluripotent state is known to be accompanied by extensive restructuring of mitochondria and switch in metabolic requirements. Here we utilized Leber's hereditary optic neuropathy (LHON) as a mitochondrial disease model to study the effects of homoplasmic mtDNA mutations and subsequent oxidative phosphorylation (OXPHOS) defects in reprogramming. We obtained fibroblasts from a total of 6 LHON patients and control subjects, and showed a significant defect in complex I respiration in LHON fibroblasts by high-resolution respiratory analysis. Using episomal vector reprogramming, our results indicated that human induced pluripotent stem cell (hiPSC) generation ..
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Grants
Awarded by Jack Brockhoff Foundation
Funding Acknowledgements
This work was supported by grants from the National Health and Medical Research Council (RW, #1084256), Australia Mitochondria Disease Foundation (RW), Retina Australia (AWH, AP, RW, SH), the Ophthalmic Research Institute of Australia (RW, SH, IAT, AWH, AP), Stafford Fox Medical Foundation (SL) and Jack Brockhoff Foundation (IAT, AH, AP); fellowships from the Cranbourne foundation (RW), NHMRC (AWH), Australian Research Council (AP, #140100047) and operational infrastructure support (Centre of Eye Research Australia, O'Brien Institute Department, St Vincent's Institute of Medical Research) from the Victorian Government.