Protein family
Protein sequence
Protein function
Catalytic activity
EC number
EC number description
2.6.1.44
Transferases;
Transferring nitrogenous groups;
Transaminases;
alanine—glyoxylate transaminase
2.6.1.51
Transferases;
Transferring nitrogenous groups;
Transaminases;
serine—pyruvate transaminase
| PDB | Resolution (Å) | PDB name |
|---|---|---|
| 1H0C | 2.5 | The crystal structure of human alanine:glyoxylate aminotransferase |
| 1J04 | 2.6 | Structural mechanism of enzyme mistargeting in hereditary kidney stone disease in vitro |
| 2YOB | 1.9 | High resolution AGXT_M structure |
| 3R9A | 2.35 | Human alanine-glyoxylate aminotransferase in complex with the TPR domain of human PEX5P |
| 4CBR | 2.3 | X-ray structure of the more stable human AGXT triple mutant (AGXT_HEM) |
| 4CBS | 2.3 | X-ray structure of quintuple mutant of human alanine glyoxylate aminotransferase, AGXT_RHEAM |
| 4I8A | 2.9 | Alanine-glyoxylate aminotransferase variant S187F |
| 4KXK | 2.9 | Alanine-glyoxylate aminotransferase variant K390A/K391A in complex with the TPR domain of human Pex5p |
| 4KYO | 2.2 | Alanine-glyoxylate aminotransferase variant K390A in complex with the TPR domain of human Pex5p |
| 5F9S | 1.7 | Crystal structure of human Alanine:Glyoxylate Aminotransferase major allele (AGT-Ma) at 1.7 Angstrom; internal aldimine with PLP in the active site |
| 5HHY | 1.7 | Structure of human Alanine:Glyoxylate Aminotransferase major allele (AGT-Ma) showing X-Ray induced reduction of PLP internal aldimine to 4'-deoxy-piridoxine-phosphate (PLR) |
| 5LUC | 1.8 | Crystal structure of the D183N variant of human Alanine:Glyoxylate Aminotransferase major allele (AGT-Ma) at 1.8 Angstrom; internal aldimine with PLP in the active site |
| 5OFY | 2.8 | Crystal structure of the D183N variant of human Alanine:Glyoxylate Aminotransferase major allele (AGT-Ma) at pH 9.0. 2.8 Ang; internal aldimine with PLP in the active site |
| 5OG0 | 2.5 | Crystal structure of human Alanine:Glyoxylate Aminotransferase major allele (AGT-Ma) at 2.5 Angstrom; internal aldimine with PLP in the active site |
| 6RV0 | 2.7 | human Alanine:Glyoxylate Aminotransferase major allele (AGT-Ma); with PMP in the active site |
| 6RV1 | 3.0 | human Alanine:Glyoxylate Aminotransferase major allele (AGT-Ma) |
| 7NS7 | 2.2 | Human L-alanine:glyoxylate aminotransferase minor allele variant: AGXT-Mi (P11L-I340M) |
| GO ontology
|
GO term | GO description |
|---|---|---|
| Biological Process | GO:0042853 | L-alanine catabolic process |
| Biological Process | GO:0019448 | L-cysteine catabolic process |
| Biological Process | GO:0006563 | L-serine metabolic process |
| Biological Process | GO:0007219 | Notch signaling pathway |
| Biological Process | GO:0019265 | glycine biosynthetic process, by transamination of glyoxylate |
| Biological Process | GO:0009436 | glyoxylate catabolic process |
| Biological Process | GO:0046487 | glyoxylate metabolic process |
| Biological Process | GO:0046724 | oxalic acid secretion |
| Molecular Function | GO:0008453 | alanine-glyoxylate transaminase activity |
| Molecular Function | GO:0016597 | amino acid binding |
| Molecular Function | GO:0042802 | identical protein binding |
| Molecular Function | GO:0042803 | protein homodimerization activity |
| Molecular Function | GO:0043621 | protein self-association |
| Molecular Function | GO:0030170 | pyridoxal phosphate binding |
| Molecular Function | GO:0004760 | serine-pyruvate transaminase activity |
| Molecular Function | GO:0008483 | transaminase activity |
| Cellular Component | GO:0005829 | cytosol |
| Cellular Component | GO:0043231 | intracellular membrane-bounded organelle |
| Cellular Component | GO:0005782 | peroxisomal matrix |
| Cellular Component | GO:0005777 | peroxisome |
| InterPro
|
InterPro name |
|---|---|
| IPR000192 | Aminotransferase class V domain |
| IPR015421 | Pyridoxal phosphate-dependent transferase, major domain |
| IPR015422 | Pyridoxal phosphate-dependent transferase, small domain |
| IPR015424 | Pyridoxal phosphate-dependent transferase |
| IPR020578 | Aminotransferase class-V, pyridoxal-phosphate binding site |
| IPR024169 | Serine-pyruvate aminotransferase/2-aminoethylphosphonate-pyruvate transaminase |
| Pfam
|
Pfam name |
|---|---|
| PF00266 | Aminotransferase class-V |
| Reactome
|
Reactome Name | Node type
|
Reactome Root | Reactome Root Name |
|---|---|---|---|---|
| R-HSA-389661 | Glyoxylate metabolism and glycine degradation | Internal node | R-HSA-1430728 | Metabolism |
| R-HSA-9033241 | Peroxisomal protein import | Internal node | R-HSA-9609507 | Protein localization |
| Location
|
ECO term
|
Pubmed |
|---|---|---|
| Peroxisome | ECO:0000269 | PubMed:10960483 |
| Peroxisome | ECO:0000269 | PubMed:12777626 |
| Peroxisome | ECO:0000269 | PubMed:1703535 |
| Peroxisome | ECO:0000269 | PubMed:23229545 |
| Peroxisome | ECO:0000269 | PubMed:24055001 |
| Peroxisome | ECO:0000269 | PubMed:26149463 |
| HPO ID
|
HPO name |
|---|---|
| HP:0000007 | Autosomal recessive inheritance |
| HP:0000010 | Recurrent urinary tract infections |
| HP:0000083 | Renal insufficiency |
| HP:0000121 | Nephrocalcinosis |
| HP:0000164 | Abnormality of the dentition |
| HP:0000488 | Retinopathy |
| HP:0000648 | Optic atrophy |
| HP:0000787 | Nephrolithiasis |
| HP:0000790 | Hematuria |
| HP:0000805 | Enuresis |
| HP:0000924 | Abnormality of the skeletal system |
| HP:0000965 | Cutis marmorata |
| HP:0001063 | Acrocyanosis |
| HP:0001138 | Optic neuropathy |
| HP:0001297 | Stroke |
| HP:0001508 | Failure to thrive |
| HP:0001678 | Atrioventricular block |
| HP:0001903 | Anemia |
| HP:0001939 | Abnormality of metabolism/homeostasis |
| HP:0001942 | Metabolic acidosis |
| HP:0001944 | Dehydration |
| HP:0002621 | Atherosclerosis |
| HP:0002653 | Bone pain |
| HP:0002756 | Pathologic fracture |
| HP:0003159 | Hyperoxaluria |
| HP:0003593 | Infantile onset |
| HP:0003761 | Calcinosis |
| HP:0003774 | Stage 5 chronic kidney disease |
| HP:0004417 | Intermittent claudication |
| HP:0004950 | Peripheral arterial stenosis |
| HP:0008672 | Calcium oxalate nephrolithiasis |
| HP:0009830 | Peripheral neuropathy |
| HP:0011001 | Increased bone mineral density |
| HP:0011021 | Abnormality of circulating enzyme level |
| HP:0011506 | Choroidal neovascularization |
| HP:0012213 | Decreased glomerular filtration rate |
| HP:0025324 | Arterial occlusion |
| HP:0025520 | Calcinosis cutis |
| HP:0030507 | Retinal crystals |
| HP:0030880 | Raynaud phenomenon |
| HP:0100518 | Dysuria |
| HP:0100758 | Gangrene |
| Disease name | MONDO ID
|
ICD10
|
ICD10 chapter
|
OMIM
|
Orphanet
|
|---|---|---|---|---|---|
| nephrocalcinosis | MONDO:0001567 | E83 | chapter4, Endocrine, nutritional and metabolic diseases | ||
| nephrocalcinosis | MONDO:0001567 | N29 | chapter14, Diseases of the genitourinary system | ||
| nephrolithiasis | MONDO:0008171 | N20 | chapter14, Diseases of the genitourinary system | ||
| primary hyperoxaluria type 1 | MONDO:0009823 | E74 | chapter4, Endocrine, nutritional and metabolic diseases | OMIM:259900 | Orphanet:93598 |