| URN | urn:agi-llid:3030 |
|---|---|
| Connectivity | 24 |
| Name | Hadha |
| Description | hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit |
| Notes | This gene encodes the alpha subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. Mutations in this gene result in trifunctional protein deficiency or LCHAD deficiency. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation. |
| Pathway | fatty acid beta degradation pathway |
|---|
| GO Molecular Function | catalytic activity |
|---|---|
| oxidoreductase activity | |
| lyase activity | |
| NAD or NADH binding | |
| coenzyme binding | |
| acyl-CoA binding | |
| acetyl-CoA C-acyltransferase activity | |
| acetyl-CoA C-acetyltransferase activity | |
| 3-hydroxyacyl-CoA dehydrogenase activity | |
| long-chain-3-hydroxyacyl-CoA dehydrogenase activity | |
| enoyl-CoA hydratase activity | |
| long-chain-enoyl-CoA hydratase activity |
| GO Cellular Component | mitochondrion |
|---|---|
| mitochondrial inner membrane | |
| mitochondrial nucleoid | |
| fatty acid beta-oxidation multienzyme complex | |
| mitochondrial matrix |
| GO Biological Process | metabolic process |
|---|---|
| oxidation reduction | |
| fatty acid metabolic process | |
| fatty acid beta-oxidation | |
| response to drug | |
| lipid metabolic process |
| Ariadne Ontology | Aromatic amino acid metabolism |
|---|---|
| Branched amino acid metabolism | |
| Asp-Lys-Thr-Met-Cys metabolsm | |
| Fatty acid oxidation |
| Group | Aromatic amino acid metabolism |
|---|---|
| Branched amino acid metabolism | |
| Asp-Lys-Thr-Met-Cys metabolsm | |
| Fatty acid oxidation | |
| catalytic activity | |
| metabolic process | |
| oxidation reduction | |
| fatty acid metabolic process | |
| fatty acid beta-oxidation | |
| response to drug | |
| lipid metabolic process | |
| oxidoreductase activity | |
| lyase activity | |
| NAD or NADH binding | |
| coenzyme binding | |
| acyl-CoA binding | |
| acetyl-CoA C-acyltransferase activity | |
| acetyl-CoA C-acetyltransferase activity | |
| 3-hydroxyacyl-CoA dehydrogenase activity | |
| long-chain-3-hydroxyacyl-CoA dehydrogenase activity | |
| enoyl-CoA hydratase activity | |
| long-chain-enoyl-CoA hydratase activity | |
| mitochondrion | |
| mitochondrial inner membrane | |
| mitochondrial nucleoid | |
| fatty acid beta-oxidation multienzyme complex | |
| mitochondrial matrix |
| MedScan ID | 3030 |
|---|
| Hugo ID | 4801 |
|---|
| Human chromosome position | 2p23 |
|---|
| LocusLink ID | 3030 |
|---|---|
| 97212 | |
| 170670 |
| Alias | GBP |
|---|---|
| ECHA | |
| HADH | |
| LCEH | |
| MTPA | |
| LCHAD | |
| MGC1728 | |
| TP-ALPHA | |
| trifunctional enzyme subunit alpha, mitochondrial | |
| 3-oxoacyl-CoA thiolase | |
| gastrin-binding protein | |
| 78 kDa gastrin-binding protein | |
| long-chain 2-enoyl-CoA hydratase | |
| long-chain-3-hydroxyacyl-CoA dehydrogenase | |
| mitochondrial trifunctional enzyme, alpha subunit | |
| mitochondrial trifunctional protein, alpha subunit | |
| 3-ketoacyl-Coenzyme A (CoA) thiolase, alpha subunit | |
| mitochondrial long-chain 2-enoyl-Coenzyme A (CoA) hydratase, alpha subunit | |
| mitochondrial long-chain L-3-hydroxyacyl-Coenzyme A (CoA) dehydrogenase, alpha subunit | |
| hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit | |
| C77020 | |
| OTTMUSP00000029769 | |
| MGC105338 | |
| RGD1560655 | |
| 3-ketoacyl-coenzyme a (coa) thiolase | |
| enoyl-Coenzyme A hydratase | |
| HADHA | |
| hydroxyacyl dehydrogenase, subunit A | |
| hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit | |
| hydroxyacyl-Coenzyme A dehydrogenase, alpha subunit | |
| LOC231085 | |
| LOC362714 | |
| long-chain hydroxyacyl-CoA dehydrogenase | |
| Mitochondrial trifunctional enzyme alpha subunit | |
| mitochondrial trifunctional protein | |
| OTTHUMP00000122457 | |
| trifunctional protein, alpha subunit | |
| Mitochondrial trifunctional enzyme alpha subunit precursor | |
| mitochondrial trifunctional, alpha subunit | |
| C77020s | |
| expressed sequence C77020 |
| Organism | Homo sapiens |
|---|---|
| Mus musculus | |
| Rattus norvegicus |
| OMIM ID | 600890 |
|---|---|
| 609016 | |
| 609015 |
| Mouse chromosome position | 5 |
|---|---|
| 5 B1 |
| GO ID | 0003857 |
|---|---|
| 0051287 | |
| 0003985 | |
| 0003988 | |
| 0000062 | |
| 0004300 | |
| 0016509 | |
| 0016508 | |
| 0016829 | |
| 0016491 | |
| 0006635 | |
| 0006631 | |
| 0006629 | |
| 0055114 | |
| 0042493 | |
| 0016507 | |
| 0005743 | |
| 0005759 | |
| 0042645 | |
| 0005739 | |
| 0003824 | |
| 0050662 | |
| 0008152 | |
| 0005488 | |
| 0005575 |
| Rat chromosome position | 6q14 |
|---|
| Swiss-Prot Accession | P40939 |
|---|---|
| Q53T69 | |
| Q53TA2 | |
| Q9UQC5 | |
| Q8BMS1 | |
| Q3TB39 | |
| Q8BKS7 | |
| Q64428 | |
| Q99521 | |
| B2R7L4 | |
| Q16679 | |
| Q96GT7 | |
| Q5BIZ5 | |
| Q3TCY3 | |
| Q5U5Y5 | |
| Q8QZU4 |
| PIR ID | JC2108 |
|---|---|
| A49681 |
| Unigene ID | Hs.516032 |
|---|---|
| Mm.200497 | |
| Rn.3340 | |
| Rn.34751 |
| KEGG ID | hsa:3030 |
|---|---|
| mmu:97212 | |
| rno:170670 |
| EC Number | 1.1.1.211 |
|---|---|
| 4.2.1.17 |
| Swiss-Prot ID | ECHA_HUMAN |
|---|---|
| ECHA_RAT | |
| ECHA_MOUSE | |
| B2R7L4_HUMAN |
| Cell Localization | Mitochondrion |
|---|---|
| Mitochondrion matrix | |
| Cytoplasm | |
| Mitochondria |
| IPI ID | IPI00031522 |
|---|---|
| IPI00212622 | |
| IPI00223092 | |
| IPI00474476 |
| Homologene ID | 152 |
|---|
| RGD ID | 620512 |
|---|
| MGI ID | 2135593 |
|---|