“MTU1 Degradation in Mitochondrial Protein Synthesis Pathology: CLPP as a Novel Therapeutic Target”

https://pubmed.ncbi.nlm.nih.gov/38113276

The study finds that pathological mutations in MTU1, a gene controlling protein synthesis in mitochondria, lead to its rapid degradation, reduced 2-thiolation of mitochondrial transfer RNAs, and poor patient prognosis, suggesting that targeting CLPP, the protease involved in MTU1 degradation, may offer a novel therapeutic strategy.