Article
Deoxyribonucleoside treatment rescues EtBr-induced mtDNA depletion in iPSC-derived neural stem cells with POLG mutations.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology - 1 Sept 2023
Kristiansen Cecilie Katrin, Furriol Jessica, Chen Anbin, Sullivan Gareth John, Bindoff Laurence A, Liang Kristina Xiao
Abstract excerpt
Mutations in POLG, the gene encoding the catalytic subunit of the mitochondrial DNA (mtDNA) polymerase gamma (Pol-γ), lead to diseases driven by defective mtDNA maintenance. Despite being the most prevalent cause of mitochondrial disease, treatments for POLG-related disorders remain elusive. In this study, we used POLG patient-induced pluripotent stem cell (iPSC)-derived neural stem cells (iNSCs), one homozygous...
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