TruB pseudouridine synthase family member 2 (TRUB2)

TruB pseudouridine synthase family member 2 (TRUB2) is a mitochondrial enzyme that catalyzes the isomerization of uridine to pseudouridine in mitochondrial mRNAs, such as COXI and COXIII, and in a subset of tRNAs—especially synthesizing pseudouridine(55) from uracil-55 in the psi GC loop of tRNAs. As part of a protein-RNA module with other mitochondrial proteins and 16S mt-rRNA, TRUB2 plays a regulatory role in 16S mt-rRNA abundance and is essential for mitochondrial translation. Genetic defects in TRUB2 have been linked to autosomal recessive spastic ataxia and primary coenzyme Q10 deficiency.

Other names

Pseudouridylate synthase TRUB2, mitochondrialTRUB2Psi55 synthase TRUB2CLONE24922TruB pseudouridine synthase homolog 2tRNA pseudouridine 55 synthase TRUB2TruB pseudouridine (psi) synthase family member 2truB pseudouridine synthase homolog 2mitochondrial mRNA pseudouridine synthase TRUB2probable tRNA pseudouridine synthase 2