Specificity of this enzyme was not studied in details. Most probably it is very similar to the specificity of its yest and mouse homologs.
Mitochondrial. Exist in two isoforms. Reduced or no activity of Pus1 is related to mitochondrial myopathy and sideroblastic anemia (MLASA).
Can modify the Steroid receptor RNA Activator (SRA).
Mitochondrial myopathy and sideroblastic anemia (MLASA)
A small RNA derived from RNA coactivator SRA blocks steroid receptor signaling via inhibition of Pus1p-mediated pseudouridylation of SRA: evidence of a novel RNA binding domain in the N-terminus of steroid receptors.
In Human Pseudouridine Synthase 1 (hPus1), a C-Terminal Helical Insert Blocks tRNA from Binding in the Same Orientation as in the Pus1 Bacterial Homologue TruA, Consistent with Their Different Target Selectivities.
Czudnochowski N, Wang AL, Finer-Moore J, Stroud RM