Protein family
Protein sequence
Protein function
Catalytic activity
EC number
EC number description
6.1.1.10
Ligases;
Forming carbon-oxygen bonds;
Ligases forming aminoacyl-tRNA and related compounds;
methionine—tRNA ligase
| PDB | Resolution (Å) | PDB name |
|---|---|---|
| 2DJV | Solution structures of the WHEP-TRS domain of human methionyl-tRNA synthetase | |
| 4BL7 | 1.892 | Crystal structure of the AIMP3-MRS N-terminal domain complex in different space group |
| 4BVX | 1.6 | Crystal structure of the AIMP3-MRS N-terminal domain complex with I3C |
| 4BVY | 1.992 | Crystal structure of the AIMP3-MRS N-terminal domain complex |
| 5GL7 | 2.013 | Crystal structure of a truncated human cytosolic methionyl-tRNA synthetase |
| 5GOY | 2.278 | The crystal structure of human cytosolic methionyl-tRNA synthetase in complex with methionine |
| 5Y6L | 2.9 | A subcomplex crystal structure of human cytosolic aspartyl-tRNA synthetase and heterotetrameric glutathione transferase-homology domains in multi-tRNA synthetase complex |
| GO ontology
|
GO term | GO description |
|---|---|---|
| Biological Process | GO:0071364 | cellular response to epidermal growth factor stimulus |
| Biological Process | GO:0036120 | cellular response to platelet-derived growth factor stimulus |
| Biological Process | GO:0006431 | methionyl-tRNA aminoacylation |
| Biological Process | GO:1901838 | positive regulation of transcription of nucleolar large rRNA by RNA polymerase I |
| Biological Process | GO:0009303 | rRNA transcription |
| Biological Process | GO:0006418 | tRNA aminoacylation for protein translation |
| Molecular Function | GO:0005524 | ATP binding |
| Molecular Function | GO:0004825 | methionine-tRNA ligase activity |
| Molecular Function | GO:0000049 | tRNA binding |
| Cellular Component | GO:0017101 | aminoacyl-tRNA synthetase multienzyme complex |
| Cellular Component | GO:0005737 | cytoplasm |
| Cellular Component | GO:0005829 | cytosol |
| Cellular Component | GO:0070062 | extracellular exosome |
| Cellular Component | GO:0016020 | membrane |
| Cellular Component | GO:0005730 | nucleolus |
| InterPro
|
InterPro name |
|---|---|
| IPR000738 | WHEP-TRS domain |
| IPR001412 | Aminoacyl-tRNA synthetase, class I, conserved site |
| IPR004046 | Glutathione S-transferase, C-terminal |
| IPR009068 | S15/NS1, RNA-binding |
| IPR009080 | Aminoacyl-tRNA synthetase, class Ia, anticodon-binding |
| IPR010987 | Glutathione S-transferase, C-terminal-like |
| IPR014729 | Rossmann-like alpha/beta/alpha sandwich fold |
| IPR014758 | Methionyl-tRNA synthetase |
| IPR015413 | Methionyl/Leucyl tRNA synthetase |
| IPR023458 | Methionine-tRNA ligase, type 1 |
| IPR029038 | Methionyl-tRNA synthetase, Zn-domain |
| IPR033911 | Methioninyl-tRNA synthetase core domain |
| IPR036282 | Glutathione S-transferase, C-terminal domain superfamily |
| IPR041598 | Methionine--tRNA ligase, N-terminal |
| IPR041872 | Methionyl-tRNA synthetase, anticodon-binding domain |
| Pfam
|
Pfam name |
|---|---|
| PF00043 | Glutathione S-transferase, C-terminal domain |
| PF00458 | WHEP-TRS domain |
| PF09334 | tRNA synthetases class I (M) |
| PF18485 | Glutathione S-transferase, N-terminal domain |
| PF19303 | Anticodon binding domain of methionyl tRNA ligase |
| Reactome
|
Reactome Name | Node type
|
Reactome Root | Reactome Root Name |
|---|---|---|---|---|
| R-HSA-2408522 | Selenoamino acid metabolism | Internal node | R-HSA-1430728 | Metabolism |
| R-HSA-379716 | Cytosolic tRNA aminoacylation | Leaf | R-HSA-392499 | Metabolism of proteins |
| Location
|
ECO term
|
Pubmed |
|---|---|---|
| Cytoplasm, cytosol | ECO:0000305 | PubMed:10791971 |
| Cytoplasm, cytosol | ECO:0000305 | PubMed:19289464 |
| Cytoplasm, cytosol | ECO:0000305 | PubMed:26472928 |
| Nucleus, nucleolus | ECO:0000269 | PubMed:10791971 |
| HPO ID
|
HPO name |
|---|---|
| HP:0000006 | Autosomal dominant inheritance |
| HP:0000007 | Autosomal recessive inheritance |
| HP:0000100 | Nephrotic syndrome |
| HP:0000286 | Epicanthus |
| HP:0000689 | Dental malocclusion |
| HP:0000821 | Hypothyroidism |
| HP:0001156 | Brachydactyly |
| HP:0001217 | Clubbing |
| HP:0001249 | Intellectual disability |
| HP:0001251 | Ataxia |
| HP:0001252 | Hypotonia |
| HP:0001256 | Intellectual disability, mild |
| HP:0001263 | Global developmental delay |
| HP:0001270 | Motor delay |
| HP:0001284 | Areflexia |
| HP:0001288 | Gait disturbance |
| HP:0001315 | Reduced tendon reflexes |
| HP:0001388 | Joint laxity |
| HP:0001394 | Cirrhosis |
| HP:0001395 | Hepatic fibrosis |
| HP:0001396 | Cholestasis |
| HP:0001397 | Hepatic steatosis |
| HP:0001399 | Hepatic failure |
| HP:0001508 | Failure to thrive |
| HP:0001894 | Thrombocytosis |
| HP:0001903 | Anemia |
| HP:0001987 | Hyperammonemia |
| HP:0002013 | Vomiting |
| HP:0002061 | Lower limb spasticity |
| HP:0002093 | Respiratory insufficiency |
| HP:0002094 | Dyspnea |
| HP:0002206 | Pulmonary fibrosis |
| HP:0002240 | Hepatomegaly |
| HP:0002355 | Difficulty walking |
| HP:0002378 | Hand tremor |
| HP:0002460 | Distal muscle weakness |
| HP:0002705 | High, narrow palate |
| HP:0002936 | Distal sensory impairment |
| HP:0003128 | Lactic acidosis |
| HP:0003355 | Aminoaciduria |
| HP:0003376 | Steppage gait |
| HP:0003477 | Peripheral axonal neuropathy |
| HP:0003584 | Late onset |
| HP:0003593 | Infantile onset |
| HP:0003596 | Middle age onset |
| HP:0003623 | Neonatal onset |
| HP:0003676 | Progressive |
| HP:0003677 | Slowly progressive |
| HP:0003693 | Distal amyotrophy |
| HP:0006517 | Intraalveolar phospholipid accumulation |
| HP:0006530 | Abnormal pulmonary interstitial morphology |
| HP:0007020 | Progressive spastic paraplegia |
| HP:0008064 | Ichthyosis |
| HP:0008070 | Sparse hair |
| HP:0008404 | Nail dystrophy |
| HP:0009027 | Foot dorsiflexor weakness |
| HP:0009130 | Hand muscle atrophy |
| HP:0009830 | Peripheral neuropathy |
| HP:0011463 | Childhood onset |
| HP:0012418 | Hypoxemia |
| HP:0012447 | Abnormal myelination |
| HP:0012735 | Cough |
| HP:0030237 | Hand muscle weakness |
| HP:0030948 | Elevated gamma-glutamyltransferase level |
| HP:0031956 | Elevated circulating aspartate aminotransferase concentration |
| HP:0031964 | Elevated circulating alanine aminotransferase concentration |
| HP:0032152 | Keratosis pilaris |
| HP:0045055 | Tiger tail banding |
| HP:0045075 | Sparse eyebrow |
| HP:0100022 | Abnormality of movement |
| Disease name | MONDO ID
|
ICD10
|
ICD10 chapter
|
OMIM
|
Orphanet
|
|---|---|---|---|---|---|
| severe early-onset pulmonary alveolar proteinosis due to mars deficiency | MONDO:0014206 | J84 | chapter10, Diseases of the respiratory system | OMIM:615486 | Orphanet:370088 |
| severe early-onset pulmonary alveolar proteinosis due to mars deficiency | MONDO:0014206 | J84 | chapter10, Diseases of the respiratory system | OMIM:615486 | Orphanet:440427 |
| charcot-marie-tooth disease axonal type 2u | MONDO:0014566 | G60 | chapter6, Diseases of the nervous system | OMIM:616280 | Orphanet:397735 |
| autosomal recessive spastic paraplegia type 70 | MONDO:0018422 | G11 | chapter6, Diseases of the nervous system | Orphanet:401835 |