Mitochondrial RNA pseudouridine synthase Rpusd4 (Rpusd4)

Catalyzes uridine to pseudouridine isomerization (pseudouridylation) of distinct mitochondrial RNA substrates. Acts on position 1397 in 16S mitochondrial ribosomal RNA (16S mt- rRNA).

This modification is required for the assembly of 16S mt- rRNA into a functional mitochondrial ribosome. Acts on position 39 in mitochondrial tRNA(Phe).