Article
A Combined Model of Human iPSC-Derived Liver Organoids and Hepatocytes Reveals Ferroptosis in DGUOK Mutant mtDNA Depletion Syndrome.
Advanced science (Weinheim, Baden-Wurttemberg, Germany) - 1 May 2021
Guo Jingyi, Duan Lifan, He Xueying, Li Shengbiao, Wu Yi, Xiang Ge, Bao Feixiang, Yang Liang, Shi Hongyan, Gao Mi, Zheng Lingjun, Hu Huili, Liu Xingguo
Abstract excerpt
Mitochondrial DNA depletion syndrome (MDS) is a group of severe inherited disorders caused by mutations in genes, such as deoxyribonucleoside kinase (DGUOK). A great majority of DGUOK mutant MDS patients develop iron overload progressing to severe liver failure. However, the pathological mechanisms connecting iron overload and hepatic damage remains uncovered. Here, two patients' skin fibroblasts are reprogrammed...
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