Protein family
Protein sequence
Protein function
Catalytic activity
EC number
EC number description
6.1.1.4
Ligases;
Forming carbon-oxygen bonds;
Ligases forming aminoacyl-tRNA and related compounds;
leucine—tRNA ligase
PDB | Resolution (Å) | PDB name |
---|---|---|
2WFD | 3.25 | Structure of the human cytosolic leucyl-tRNA synthetase editing domain |
6KID | 3.15 | Crystal structure of human leucyl-tRNA synthetase, ATP-bound form |
6KIE | 3.15 | Crystal structure of human leucyl-tRNA synthetase, Leu-AMS-bound form |
6KQY | 3.3 | Crystal structure of human leucyl-tRNA synthetase, Leucine-bound form |
6KR7 | 4.0 | Crystal structure of methylated human leucyl-tRNA synthetase, Leu-AMS-bound form |
6LPF | 2.49 | The crystal structure of human cytoplasmic LRS |
6LR6 | 3.009 | The crystal structure of human cytoplasmic LRS |
GO ontology | GO term | GO description |
---|---|---|
Biological Process | GO:0034198 | cellular response to amino acid starvation |
Biological Process | GO:0071230 | cellular response to amino acid stimulus |
Biological Process | GO:0071233 | cellular response to leucine |
Biological Process | GO:1990253 | cellular response to leucine starvation |
Biological Process | GO:0006425 | glutaminyl-tRNA aminoacylation |
Biological Process | GO:0006429 | leucyl-tRNA aminoacylation |
Biological Process | GO:0043547 | positive regulation of GTPase activity |
Biological Process | GO:0032008 | positive regulation of TOR signaling |
Biological Process | GO:1904263 | positive regulation of TORC1 signaling |
Biological Process | GO:0008361 | regulation of cell size |
Biological Process | GO:0006418 | tRNA aminoacylation for protein translation |
Molecular Function | GO:0005524 | ATP binding |
Molecular Function | GO:0005096 | GTPase activator activity |
Molecular Function | GO:0002161 | aminoacyl-tRNA editing activity |
Molecular Function | GO:0004819 | glutamine-tRNA ligase activity |
Molecular Function | GO:0004823 | leucine-tRNA ligase activity |
Cellular Component | GO:0017101 | aminoacyl-tRNA synthetase multienzyme complex |
Cellular Component | GO:0005737 | cytoplasm |
Cellular Component | GO:0005829 | cytosol |
Cellular Component | GO:0012505 | endomembrane system |
Cellular Component | GO:0005783 | endoplasmic reticulum |
Cellular Component | GO:0005764 | lysosome |
Cellular Component | GO:0016604 | nuclear body |
InterPro | InterPro name |
---|---|
IPR001412 | Aminoacyl-tRNA synthetase, class I, conserved site |
IPR002300 | Aminoacyl-tRNA synthetase, class Ia |
IPR004493 | Leucyl-tRNA synthetase, class Ia, archaeal/eukaryotic cytosolic |
IPR009008 | Valyl/Leucyl/Isoleucyl-tRNA synthetase, editing domain |
IPR009080 | Aminoacyl-tRNA synthetase, class Ia, anticodon-binding |
IPR013155 | Methionyl/Valyl/Leucyl/Isoleucyl-tRNA synthetase, anticodon-binding |
IPR014729 | Rossmann-like alpha/beta/alpha sandwich fold |
Pfam | Pfam name |
---|---|
PF00133 | tRNA synthetases class I (I, L, M and V) |
PF08264 | Anticodon-binding domain of 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 | ECO:0000250 |
HPO ID | HPO name |
---|---|
HP:0000007 | Autosomal recessive inheritance |
HP:0000252 | Microcephaly |
HP:0000293 | Full cheeks |
HP:0000407 | Sensorineural hearing impairment |
HP:0001250 | Seizure |
HP:0001252 | Hypotonia |
HP:0001263 | Global developmental delay |
HP:0001290 | Generalized hypotonia |
HP:0001397 | Hepatic steatosis |
HP:0001508 | Failure to thrive |
HP:0001903 | Anemia |
HP:0001972 | Macrocytic anemia |
HP:0002007 | Frontal bossing |
HP:0002194 | Delayed gross motor development |
HP:0002240 | Hepatomegaly |
HP:0002910 | Elevated hepatic transaminase |
HP:0003128 | Lactic acidosis |
HP:0003256 | Abnormality of the coagulation cascade |
HP:0006554 | Acute hepatic failure |
HP:0010511 | Long toe |
HP:0100807 | Long fingers |
Disease name | MONDO ID | ICD10 | ICD10 chapter | OMIM | Orphanet |
---|---|---|---|---|---|
infantile liver failure syndrome 1 | MONDO:0024568 | K72 | chapter11, Diseases of the digestive system | OMIM:615438 | Orphanet:370088 |