Ontology highlight
ABSTRACT:
SUBMITTER: Jing R
PROVIDER: S-EPMC6289059 | biostudies-literature | 2018 Nov
REPOSITORIES: biostudies-literature
Jing Ran R Corbett James L JL Cai Jun J Beeson Gyda C GC Beeson Craig C CC Chan Sherine S SS Dimmock David P DP Lazcares Lynn L Geurts Aron M AM Lemasters John J JJ Duncan Stephen A SA
Cell reports 20181101 6
Patients with mtDNA depletion syndrome 3 (MTDPS3) often die as children from liver failure caused by severe reduction in mtDNA content. The identification of treatments has been impeded by an inability to culture and manipulate MTDPS3 primary hepatocytes. Here we generated DGUOK-deficient hepatocyte-like cells using induced pluripotent stem cells (iPSCs) and used them to identify drugs that could improve mitochondrial ATP production and mitochondrial function. Nicotinamide adenine dinucleotide ( ...[more]