Pantethine taurine vitamin c acetaldehyde11/27/2023 ![]() Mitochondrial DNA (mtDNA) encodes 13 proteins related to the OXPHOS system, two ribosomal RNAs, and 22 transfer RNAs, which are used for mitochondrial RNA translation. Mitochondrial defects affect various metabolic pathways, including oxidative phosphorylation (OXPHOS) however, the associated changes in various metabolite levels and the exact mechanisms of mitochondrial disorders are not fully understood. Mitochondria play a key role in metabolism, and their impairments cause various diseases, ranging from specific mitochondrial disorders to aging-related diseases, such as age-related macular degeneration (AMD). Because all these phenomena were sensitive to taurine treatment, we conclude that administration of taurine may be a potential new therapeutic approach for mitochondria-related retinal diseases. Therefore, mitochondrial dysfunction caused metabolic changes, accumulation of oxidative stress that depleted GSH, and EMT in the RPE that could be involved in retinal pathogenesis. We also analyzed the retinal pigment epithelium (RPE) differentiated from MELAS iPSCs by using a three-dimensional culture system and found that it showed epithelial mesenchymal transition (EMT), which was suppressed by taurine. Taurine normalized these changes, suggesting that MELAS iPSCs were affected by the oxidative stress and taurine reduced its influence. Metabolomic analyses showed that the ratio of the reduced (GSH) to oxidized glutathione (GSSG) was decreased whereas the levels of cysteine, a substrate of GSH, and oxidative stress markers were upregulated in MELAS iPSCs. ![]() These deficits were rescued by taurine administration. Here, we found that induced pluripotent stem cells (iPSCs) from a patient with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) showed attenuated proliferation and survival when glycolysis was inhibited. However, the metabolic changes and mechanisms of mitochondrial disorders are not fully understood. ![]() Mitochondria participate in various metabolic pathways, and their dysregulation results in multiple disorders, including aging-related diseases.
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