Full name: tRNA pseudouridine synthase 1, mitochondrial
GI: 70166645
Orf: PP8985
COG: COG0101
UniProt: Q9Y606
Structures: | 4J37 | 4ITS | 4IQM | 4NZ7 | 4NZ6 |
Enzyme type: pseudouridine synthase
Position of modification - modification: t:28 - Y
t:27 - Y
other:236 - Y
t:30 - Y


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).

Protein sequence:


Enzymatic activities:

Reaction Substrate Type Position
U:Y tRNA (t) Lys/∃UU/mitochondrial 27
U:Y tRNA (t) Lys/∃UU/mitochondrial 28
U:Y tRNA (t) Ile/AAU/cytosolic 27
U:Y tRNA (t) Ile/AAU/cytosolic 30
U:Y tRNA (t) Ser/UGA/cytosolic 28
U:Y other (o) SRA//nucleoplasmic 236


Title Authors Journal Details PubMed Id DOI
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. Ghosh SK, Patton JR, Spanjaard RA Biochemistry [details] 22998747 -
Pseudouridine synthase 1: a site-specific synthase without strict sequence recognition requirements. Sibert BS, Patton JR Nucleic Acids Res [details] 22102571 -
Partial activity is seen with many substitutions of highly conserved active site residues in human Pseudouridine synthase 1. Sibert BS, Fischel-Ghodsian N, Patton JR RNA [details] 18648068 -
Mitochondrial myopathy and sideroblastic anemia (MLASA): missense mutation in the pseudouridine synthase 1 (PUS1) gene is associated with the loss of tRNA pseudouridylation. Patton JR, Bykhovskaya Y, Mengesha E, Bertolotto C, Fischel-Ghodsian N J Biol Chem [details] 15772074 -
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 J Mol Biol [details] 23707380 -
Steroid receptor RNA activator (SRA) modification by the human pseudouridine synthase 1 (hPus1p): RNA binding, activity, and atomic model. Huet T, Miannay FA, Patton JR, Thore S... PLoS One [details] 24722331 -


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