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[2] [3] There are two distinct phases in the pathway

In human, there is a set of tRNA thiolation enzymes such as mitochondrial tRNA-specific 2-thiouridylase 1 (MTU1) and cytoplasmic tRNA 2-thiolation protein 1/2 (CTU1/2), catalyzing the sulfur modification on tRNAs 40,41,42

These disruptions are associated with disease subtypes, progression rates, and clinical outcomes (334, 335, 338354, 358), and studies have shown that iron chelation therapy can delay motor neuron degeneration and extend survival in ALS mouse models (352, 355, 358) ( Figure 8 )