Protein Production Mechanism in Mitochondria (IMAGE)
Caption
In mitochondria of normal cells, protein is synthesized by taurine binding with tRNA, and many proteins are transported from the cytoplasm to mitochondria for normal functionality. However, when taurine's activity declines, as in patients with MELAS or MERRF, protein synthesis in the mitochondria stops and the mitochondrial structure collapses. Proteins are then unable to enter the mitochondria from the cytoplasm so they break down and become aggregates that cause cell damage. When TUDCA is administered, abnormal protein accumulation is suppressed and cell function is restored.
Credit
Associate Professor Wei Fan-Yan
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