Determining the cause of difficult-to-control mitochondrial diseases

A Japanese research group has discovered that taurine is conjugated with mitochondrial transfer RNAs (tRNAs), small RNAs that decode genetic information, and that taurine deficiency in tRNA dramatically reduces both mitochondrial protein translation and impairs mitochondrial membrane integrity. Subsequently, many mitochondrial proteins are unable to localize on mitochondria and end up forming toxic aggregates inside the cell. Furthermore, researchers also found that some symptoms of mitochondrial dysfunction can be improved by maintaining protein quality.