Protein family
Protein sequence
Protein function
Catalytic activity
EC number
EC number description
2.7.7.-
Transferases;
Transferring phosphorus-containing groups;
Nucleotidyltransferases;
6.1.1.14
Ligases;
Forming carbon-oxygen bonds;
Ligases forming aminoacyl-tRNA and related compounds;
glycine—tRNA ligase
| PDB | Resolution (Å) | PDB name |
|---|---|---|
| 2PME | 2.9 | The Apo crystal Structure of the glycyl-tRNA synthetase |
| 2PMF | 2.85 | The crystal structure of a human glycyl-tRNA synthetase mutant |
| 2Q5H | 3.0 | Crystal structure of apo-wildtype Glycyl-tRNA synthetase |
| 2Q5I | 2.8 | Crystal structure of apo S581L Glycyl-tRNA synthetase mutant |
| 2ZT5 | 2.5 | Crystal structure of human glycyl-trna synthetase (GLYRS) in complex with AP4A (cocrystallized with ATP) |
| 2ZT6 | 3.08 | Crystal structure of human Glycyl-tRNA synthetase (GlyRS) in complex with AMPCPP |
| 2ZT7 | 2.7 | Crystal structure of human Glycyl-tRNA synthetase (GlyRS) in complex with Glycine and ATP |
| 2ZT8 | 3.35 | Crystal structure of human Glycyl-tRNA synthetase (GlyRS) in complex with Gly-AMP analog |
| 2ZXF | 3.4 | Crystal structure of human glycyl-trna synthetase (GLYRS) in complex with AP4A (cocrystallized with AP4A) |
| 4KQE | 2.739 | The mutant structure of the human glycyl-tRNA synthetase E71G |
| 4KR2 | 3.292 | Glycyl-tRNA synthetase in complex with tRNA-Gly |
| 4KR3 | 3.235 | Glycyl-tRNA synthetase mutant E71G in complex with tRNA-Gly |
| 4QEI | 2.875 | Two distinct conformational states of GlyRS captured in crystal lattice |
| 5E6M | 2.927 | Crystal structure of human wild type GlyRS bound with tRNAGly |
| GO ontology
|
GO term | GO description |
|---|---|---|
| Biological Process | GO:0015966 | diadenosine tetraphosphate biosynthetic process |
| Biological Process | GO:0070150 | mitochondrial glycyl-tRNA aminoacylation |
| Biological Process | GO:0006418 | tRNA aminoacylation for protein translation |
| Molecular Function | GO:0005524 | ATP binding |
| Molecular Function | GO:0004081 | bis(5'-nucleosyl)-tetraphosphatase (asymmetrical) activity |
| Molecular Function | GO:0004820 | glycine-tRNA ligase activity |
| Molecular Function | GO:0042802 | identical protein binding |
| Molecular Function | GO:0046983 | protein dimerization activity |
| Molecular Function | GO:0016740 | transferase activity |
| Cellular Component | GO:0030424 | axon |
| Cellular Component | GO:0005737 | cytoplasm |
| Cellular Component | GO:0005829 | cytosol |
| Cellular Component | GO:0070062 | extracellular exosome |
| Cellular Component | GO:0005759 | mitochondrial matrix |
| Cellular Component | GO:0005739 | mitochondrion |
| Cellular Component | GO:0030141 | secretory granule |
| InterPro
|
InterPro name |
|---|---|
| IPR000738 | WHEP-TRS domain |
| IPR002314 | Aminoacyl-tRNA synthetase, class II (G/ P/ S/T) |
| IPR002315 | Glycyl-tRNA synthetase |
| IPR004154 | Anticodon-binding |
| IPR006195 | Aminoacyl-tRNA synthetase, class II |
| IPR009068 | S15/NS1, RNA-binding |
| IPR027031 | Glycyl-tRNA synthetase/DNA polymerase subunit gamma-2 |
| IPR033731 | Glycyl-tRNA synthetase-like core domain |
| IPR036621 | Anticodon-binding domain superfamily |
| IPR045864 | Class II Aminoacyl-tRNA synthetase/Biotinyl protein ligase (BPL) and lipoyl protein ligase (LPL) |
| Pfam
|
Pfam name |
|---|---|
| PF00458 | WHEP-TRS domain |
| PF00587 | tRNA synthetase class II core domain (G, H, P, S and T) |
| PF03129 | Anticodon binding domain |
| Reactome
|
Reactome Name | Node type
|
Reactome Root | Reactome Root Name |
|---|---|---|---|---|
| R-HSA-379716 | Cytosolic tRNA aminoacylation | Leaf | R-HSA-392499 | Metabolism of proteins |
| R-HSA-379726 | Mitochondrial tRNA aminoacylation | Leaf | R-HSA-392499 | Metabolism of proteins |
| Location
|
ECO term
|
Pubmed |
|---|---|---|
| Cell projection, axon | ECO:0000269 | PubMed:17035524 |
| Cell projection, axon | ECO:0000269 | PubMed:25168514 |
| Cytoplasm | ECO:0000269 | PubMed:17035524 |
| Cytoplasm | ECO:0000269 | PubMed:17529987 |
| Cytoplasm | ECO:0000269 | PubMed:26327585 |
| Mitochondrion | ECO:0000269 | PubMed:17529987 |
| Mitochondrion | ECO:0000269 | PubMed:26327585 |
| Secreted | ECO:0000250 | |
| Secreted, extracellular exosome | ECO:0000250 |
| HPO ID
|
HPO name |
|---|---|
| HP:0000006 | Autosomal dominant inheritance |
| HP:0001265 | Hyporeflexia |
| HP:0001270 | Motor delay |
| HP:0001284 | Areflexia |
| HP:0001290 | Generalized hypotonia |
| HP:0001324 | Muscle weakness |
| HP:0001347 | Hyperreflexia |
| HP:0001621 | Weak voice |
| HP:0001761 | Pes cavus |
| HP:0001763 | Pes planus |
| HP:0001765 | Hammertoe |
| HP:0002093 | Respiratory insufficiency |
| HP:0002172 | Postural instability |
| HP:0002317 | Unsteady gait |
| HP:0002460 | Distal muscle weakness |
| HP:0002495 | Impaired vibratory sensation |
| HP:0002650 | Scoliosis |
| HP:0002936 | Distal sensory impairment |
| HP:0002938 | Lumbar hyperlordosis |
| HP:0003273 | Hip contracture |
| HP:0003392 | First dorsal interossei muscle weakness |
| HP:0003393 | Thenar muscle atrophy |
| HP:0003426 | First dorsal interossei muscle atrophy |
| HP:0003427 | Thenar muscle weakness |
| HP:0003435 | Cold-induced hand cramps |
| HP:0003484 | Upper limb muscle weakness |
| HP:0003557 | Increased variability in muscle fiber diameter |
| HP:0003593 | Infantile onset |
| HP:0003677 | Slowly progressive |
| HP:0003693 | Distal amyotrophy |
| HP:0003803 | Type 1 muscle fiber predominance |
| HP:0004322 | Short stature |
| HP:0007178 | Motor polyneuropathy |
| HP:0007340 | Lower limb muscle weakness |
| HP:0008081 | Pes valgus |
| HP:0009053 | Distal lower limb muscle weakness |
| HP:0009129 | Upper limb amyotrophy |
| HP:0009830 | Peripheral neuropathy |
| HP:0010546 | Muscle fibrillation |
| HP:0011462 | Young adult onset |
| HP:0031936 | Delayed ability to walk |
| HP:0040131 | Abnormal motor nerve conduction velocity |
| Disease name | MONDO ID
|
ICD10
|
ICD10 chapter
|
OMIM
|
Orphanet
|
|---|---|---|---|---|---|
| charcot-marie-tooth disease type 2d | MONDO:0011091 | G60 | chapter6, Diseases of the nervous system | OMIM:601472 | Orphanet:99938 |
| neuronopathy, distal hereditary motor, type 5a | MONDO:0015353 | G12 | chapter6, Diseases of the nervous system | OMIM:600794 | |
| spinal muscular atrophy- infantile- james type | MONDO:0033621 | G12 | chapter6, Diseases of the nervous system | OMIM:619042 |