Protein family
Protein sequence
Protein function
Catalytic activity
EC number
EC number description
2.1.1.-
Transferases;
Transferring one-carbon groups;
Methyltransferases;
2.1.1.218
Transferases;
Transferring one-carbon groups;
Methyltransferases;
tRNA (adenine9-N1)-methyltransferase
2.1.1.221
Transferases;
Transferring one-carbon groups;
Methyltransferases;
tRNA (guanine9-N1)-methyltransferase
GO ontology | GO term | GO description |
---|---|---|
Biological Process | GO:0080009 | mRNA methylation |
Biological Process | GO:0000964 | mitochondrial RNA 5'-end processing |
Biological Process | GO:1990180 | mitochondrial tRNA 3'-end processing |
Biological Process | GO:0097745 | mitochondrial tRNA 5'-end processing |
Biological Process | GO:0070901 | mitochondrial tRNA methylation |
Biological Process | GO:0090646 | mitochondrial tRNA processing |
Biological Process | GO:0070131 | positive regulation of mitochondrial translation |
Molecular Function | GO:0003723 | RNA binding |
Molecular Function | GO:0042802 | identical protein binding |
Molecular Function | GO:0061953 | mRNA (adenine-N1-)-methyltransferase activity |
Molecular Function | GO:0016429 | tRNA (adenine-N1-)-methyltransferase activity |
Molecular Function | GO:0052905 | tRNA (guanine(9)-N(1))-methyltransferase activity |
Molecular Function | GO:0009019 | tRNA (guanine-N1-)-methyltransferase activity |
Molecular Function | GO:0000049 | tRNA binding |
Cellular Component | GO:0005759 | mitochondrial matrix |
Cellular Component | GO:0042645 | mitochondrial nucleoid |
Cellular Component | GO:0030678 | mitochondrial ribonuclease P complex |
Cellular Component | GO:0005739 | mitochondrion |
Cellular Component | GO:0005654 | nucleoplasm |
Cellular Component | GO:0005634 | nucleus |
Cellular Component | GO:0043527 | tRNA methyltransferase complex |
InterPro | InterPro name |
---|---|
IPR007356 | tRNA (guanine-N1-)-methyltransferase, eukaryotic |
IPR016009 | tRNA methyltransferase TRMD/TRM10-type domain |
IPR025812 | tRNA methyltransferase 10 homologue C, SAM-dependent MTase TRM10-type |
IPR028564 | tRNA methyltransferase TRM10-type domain |
IPR038459 | tRNA methyltransferase TRM10-type domain superfamily |
Pfam | Pfam name |
---|---|
PF01746 | tRNA (Guanine-1)-methyltransferase |
Reactome | Reactome Name | Node type | Reactome Root | Reactome Root Name |
---|---|---|---|---|
R-HSA-6785470 | tRNA processing in the mitochondrion | Leaf | R-HSA-8953854 | Metabolism of RNA |
R-HSA-6787450 | tRNA modification in the mitochondrion | Leaf | R-HSA-8953854 | Metabolism of RNA |
R-HSA-8868766 | rRNA processing in the mitochondrion | Internal node | R-HSA-8953854 | Metabolism of RNA |
Location | ECO term | Pubmed |
---|---|---|
Mitochondrion matrix, mitochondrion nucleoid | ECO:0000269 | PubMed:18984158 |
Mitochondrion matrix, mitochondrion nucleoid | ECO:0000269 | PubMed:23473034 |
Mitochondrion matrix, mitochondrion nucleoid | ECO:0000269 | PubMed:24703694 |
HPO ID | HPO name |
---|---|
HP:0000007 | Autosomal recessive inheritance |
HP:0000407 | Sensorineural hearing impairment |
HP:0001252 | Hypotonia |
HP:0001410 | Decreased liver function |
HP:0001508 | Failure to thrive |
HP:0001522 | Death in infancy |
HP:0001712 | Left ventricular hypertrophy |
HP:0002020 | Gastroesophageal reflux |
HP:0002033 | Poor suck |
HP:0002098 | Respiratory distress |
HP:0002126 | Polymicrogyria |
HP:0002151 | Increased serum lactate |
HP:0002490 | Increased CSF lactate |
HP:0003128 | Lactic acidosis |
HP:0003348 | Hyperalaninemia |
HP:0003623 | Neonatal onset |
HP:0011968 | Feeding difficulties |
HP:0030948 | Elevated gamma-glutamyltransferase level |
HP:0031956 | Elevated circulating aspartate aminotransferase concentration |
HP:0031964 | Elevated circulating alanine aminotransferase concentration |
HP:0032653 | Elevated lactate:pyruvate ratio |
Disease name | MONDO ID | ICD10 | ICD10 chapter | OMIM | Orphanet |
---|---|---|---|---|---|
combined oxidative phosphorylation defect type 30 | MONDO:0014856 | G93 | chapter6, Diseases of the nervous system | OMIM:616974 | Orphanet:478042 |