UniProt functional annotation for O15091

UniProt code: O15091.

Organism: Homo sapiens (Human).
Taxonomy: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo.
 
Function: Catalytic ribonuclease component of mitochondrial ribonuclease P, a complex composed of TRMT10C/MRPP1, HSD17B10/MRPP2 and PRORP/MRPP3, which cleaves tRNA molecules in their 5'-ends (PubMed:18984158, PubMed:25953853). The presence of TRMT10C/MRPP1, HSD17B10/MRPP2 is required to catalyze tRNA molecules in their 5'-ends (PubMed:25953853). {ECO:0000269|PubMed:18984158, ECO:0000269|PubMed:25953853}.
 
Catalytic activity: Reaction=Endonucleolytic cleavage of RNA, removing 5'-extranucleotides from tRNA precursor.; EC=3.1.26.5; Evidence={ECO:0000269|PubMed:25953853};
Cofactor: Name=Mg(2+); Xref=ChEBI:CHEBI:18420; Evidence={ECO:0000305|PubMed:25953853}; Name=Mn(2+); Xref=ChEBI:CHEBI:29035; Evidence={ECO:0000305|PubMed:25953853}; Note=Binds 2 Mg(2+) or Mg(2+) ions per subunit. {ECO:0000305|PubMed:25953853};
Subunit: Catalytic component of mitochondrial ribonuclease P, a complex composed of TRMT10C/MRPP1, HSD17B10/MRPP2 and PRORP/MRPP3 (PubMed:18984158). {ECO:0000269|PubMed:18984158}.
Subcellular location: Mitochondrion {ECO:0000269|PubMed:18984158}.
Induction: Down-regulated following mitochondrial unfolded protein response. {ECO:0000269|PubMed:27350246}.
Domain: Displays a distorted and non-productive active site that probably switches to a fully productive state only upon association with TRMT10C/MRPP1, HSD17B10/MRPP2 and pre-tRNA substrate. {ECO:0000305|PubMed:25953853}.
Ptm: Degraded by LONP1 following mitochondrial unfolded protein response, probably leading to inhibit translation in mitochondrion. {ECO:0000269|PubMed:27350246}.
Similarity: Belongs to the PPR family. P subfamily. {ECO:0000305}.
Sequence caution: Sequence=BAA20845.2; Type=Erroneous initiation; Evidence={ECO:0000305};

Annotations taken from UniProtKB at the EBI.