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 |