TruB pseudouridine synthase family member 1 (TRUB1)

TruB pseudouridine synthase family member 1 (TRUB1) is a highly conserved enzyme responsible for catalyzing the formation of pseudouridine (Ψ) at position 55 in specific mitochondrial tRNAs, a key modification that ensures proper RNA structure and mitochondrial translation. TRUB1 is widely expressed in human tissues and localizes primarily to the nucleus with additional presence in mitochondria. Beyond its canonical enzyme function in tRNA modification, TRUB1 also regulates maturation of let-7 microRNA family members independently of its enzymatic activity, by directly binding to primary miRNA transcripts and promoting their processing. While dysfunction of TRUB1 is linked to defects in mitochondrial protein synthesis and has been associated with rare mitochondrial diseases, there are currently no drugs that directly target TRUB1, and it is not considered a classic therapeutic target for drug development.

Other names

TRUB1tRNA pseudouridine(55) synthasepseudouridine synthase 1psi55 synthase homolog (E. coli)human tRNA Psi55-synthase