Protein family
Protein sequence
Protein function
Publications
Catalytic activity
EC number
EC number description
2.7.11.-
Transferases;
Transferring phosphorus-containing groups;
Protein-serine/threonine kinases;
4.1.1.32
Lyases;
Carbon-carbon lyases;
Carboxy-lyases;
phosphoenolpyruvate carboxykinase (GTP)
| PDB | Resolution (Å) | PDB name |
|---|---|---|
| 1KHB | 1.854 | PEPCK complex with nonhydrolyzable GTP analog, native data |
| 1KHE | 2.4 | PEPCK complex with nonhydrolyzable GTP analog, MAD data |
| 1KHF | 2.02 | PEPCK complex with PEP |
| 1KHG | 2.34 | PEPCK |
| 1M51 | 2.25 | PEPCK complex with a GTP-competitive inhibitor |
| 1NHX | 2.1 | PEPCK COMPLEX WITH A GTP-COMPETITIVE INHIBITOR |
| 2GMV | 2.3 | PEPCK complex with a GTP-competitive inhibitor |
GO ontology
|
GO term | GO description |
|---|---|---|
| Biological Process | GO:0071549 | cellular response to dexamethasone stimulus |
| Biological Process | GO:0071333 | cellular response to glucose stimulus |
| Biological Process | GO:0032869 | cellular response to insulin stimulus |
| Biological Process | GO:0051365 | cellular response to potassium ion starvation |
| Biological Process | GO:0006094 | gluconeogenesis |
| Biological Process | GO:0042593 | glucose homeostasis |
| Biological Process | GO:0006006 | glucose metabolic process |
| Biological Process | GO:0046327 | glycerol biosynthetic process from pyruvate |
| Biological Process | GO:0070365 | hepatocyte differentiation |
| Biological Process | GO:0006107 | oxaloacetate metabolic process |
| Biological Process | GO:0018105 | peptidyl-serine phosphorylation |
| Biological Process | GO:0046889 | positive regulation of lipid biosynthetic process |
| Biological Process | GO:0043382 | positive regulation of memory T cell differentiation |
| Biological Process | GO:0061402 | positive regulation of transcription from RNA polymerase II promoter in response to acidic pH |
| Biological Process | GO:0019543 | propionate catabolic process |
| Biological Process | GO:0046890 | regulation of lipid biosynthetic process |
| Biological Process | GO:0009617 | response to bacterium |
| Biological Process | GO:0032868 | response to insulin |
| Biological Process | GO:0042594 | response to starvation |
| Biological Process | GO:0072350 | tricarboxylic acid metabolic process |
| Molecular Function | GO:0005525 | GTP binding |
| Molecular Function | GO:0031406 | carboxylic acid binding |
| Molecular Function | GO:0000287 | magnesium ion binding |
| Molecular Function | GO:0030145 | manganese ion binding |
| Molecular Function | GO:0004613 | phosphoenolpyruvate carboxykinase (GTP) activity |
| Molecular Function | GO:0106264 | protein serine kinase activity (using GTP as donor) |
| Cellular Component | GO:0005737 | cytoplasm |
| Cellular Component | GO:0005829 | cytosol |
| Cellular Component | GO:0005783 | endoplasmic reticulum |
| Cellular Component | GO:0070062 | extracellular exosome |
| InterPro
|
InterPro name |
|---|---|
| IPR008209 | Phosphoenolpyruvate carboxykinase, GTP-utilising |
| IPR008210 | Phosphoenolpyruvate carboxykinase, N-terminal |
| IPR013035 | Phosphoenolpyruvate carboxykinase, C-terminal |
| IPR018091 | Phosphoenolpyruvate carboxykinase, GTP-utilising, conserved site |
| IPR035077 | Phosphoenolpyruvate carboxykinase, C-terminal P-loop domain |
| IPR035078 | Phosphoenolpyruvate carboxykinase, GTP-utilising, N-terminal |
| Pfam
|
Pfam name |
|---|---|
| PF00821 | Phosphoenolpyruvate carboxykinase C-terminal P-loop domain |
| PF17297 | Phosphoenolpyruvate carboxykinase N-terminal domain |
| Reactome
|
Reactome Name | Node type
|
Reactome Root | Reactome Root Name |
|---|---|---|---|---|
| R-HSA-2161541 | Abacavir metabolism | Leaf | R-HSA-9748784 | Drug ADME |
| R-HSA-381340 | Transcriptional regulation of white adipocyte differentiation | Leaf | R-HSA-1266738 | Developmental Biology |
| R-HSA-70263 | Gluconeogenesis | Leaf | R-HSA-1430728 | Metabolism |
| R-HSA-9615017 | FOXO-mediated transcription of oxidative stress, metabolic and neuronal genes | Leaf | R-HSA-74160 | Gene expression (Transcription) |
| R-HSA-9632974 | NR1H2 & NR1H3 regulate gene expression linked to gluconeogenesis | Leaf | R-HSA-162582 | Signal Transduction |
| Location
|
ECO term
|
Pubmed |
|---|---|---|
| Cytoplasm, cytosol | ECO:0000269 | PubMed:32322062 |
| Endoplasmic reticulum | ECO:0000269 | PubMed:32322062 |
| HPO ID
|
HPO name |
|---|---|
| HP:0000007 | Autosomal recessive inheritance |
| HP:0000648 | Optic atrophy |
| HP:0000799 | Renal steatosis |
| HP:0000961 | Cyanosis |
| HP:0001250 | Seizure |
| HP:0001263 | Global developmental delay |
| HP:0001397 | Hepatic steatosis |
| HP:0001399 | Hepatic failure |
| HP:0001943 | Hypoglycemia |
| HP:0002059 | Cerebral atrophy |
| HP:0002104 | Apnea |
| HP:0002240 | Hepatomegaly |
| HP:0002353 | EEG abnormality |
| HP:0002480 | Hepatic encephalopathy |
| HP:0002919 | Ketonuria |
| HP:0003128 | Lactic acidosis |
| HP:0003162 | Fasting hypoglycemia |
| HP:0003572 | Low plasma citrulline |
| HP:0003593 | Infantile onset |
| HP:0005959 | Impaired gluconeogenesis |
| HP:0031141 | Increased hepatic echogenicity |
| HP:0031964 | Elevated circulating alanine aminotransferase concentration |
| Tissue
|
Cell type
|
Expression level | Reliability |
|---|---|---|---|
| adipose tissue | adipocytes | Low | Approved |
| appendix | glandular cells | Medium | Approved |
| breast | adipocytes | Low | Approved |
| colon | glandular cells | Medium | Approved |
| duodenum | glandular cells | Medium | Approved |
| gallbladder | glandular cells | Low | Approved |
| kidney | cells in tubules | High | Approved |
| liver | cholangiocytes | Low | Approved |
| liver | hepatocytes | Low | Approved |
| rectum | glandular cells | Medium | Approved |
| small intestine | glandular cells | Medium | Approved |
| stomach 1 | glandular cells | Low | Approved |
| stomach 2 | glandular cells | Low | Approved |
| vagina | squamous epithelial cells | Low | Approved |
| Disease name | MONDO ID
|
ICD10
|
ICD10 chapter
|
OMIM
|
Orphanet
|
|---|---|---|---|---|---|
| phosphoenolpyruvate carboxykinase deficiency, cytosolic | MONDO:0009866 | E74 | chapter4, Endocrine, nutritional and metabolic diseases | OMIM:261680 | Orphanet:79316 |