| URN | urn:agi-llid:3032 |
|---|---|
| Connectivity | 7 |
| Name | Hadhb |
| Description | hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit |
| Notes | This gene encodes the beta 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 beta subunit catalyzing the 3-ketoacyl-CoA thiolase activity. Mutations in this gene result in trifunctional protein deficiency. The encoded protein can also bind RNA and decreases the stability of some mRNAs. 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. Alternatively spliced transcript variants have been found; however, their full-length nature is not known. |
| Pathway | fatty acid beta degradation pathway |
|---|
| GO Molecular Function | catalytic activity |
|---|---|
| transferase activity | |
| NAD or NADH binding | |
| acyl-CoA binding | |
| transferase activity, transferring acyl groups other than amino-acyl groups | |
| acyltransferase activity | |
| acetyl-CoA C-acyltransferase 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 | |
| mitochondrial envelope | |
| fatty acid beta-oxidation multienzyme complex | |
| mitochondrial matrix |
| GO Biological Process | metabolic process |
|---|---|
| fatty acid metabolic process | |
| fatty acid beta-oxidation | |
| lipid metabolic process |
| Ariadne Ontology | Branched amino acid metabolism |
|---|---|
| Fatty acid oxidation |
| Group | Branched amino acid metabolism |
|---|---|
| Fatty acid oxidation | |
| catalytic activity | |
| metabolic process | |
| fatty acid metabolic process | |
| fatty acid beta-oxidation | |
| lipid metabolic process | |
| transferase activity | |
| NAD or NADH binding | |
| acyl-CoA binding | |
| transferase activity, transferring acyl groups other than amino-acyl groups | |
| acyltransferase activity | |
| acetyl-CoA C-acyltransferase 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 | |
| mitochondrial envelope | |
| fatty acid beta-oxidation multienzyme complex | |
| mitochondrial matrix |
| MedScan ID | 3032 |
|---|
| Hugo ID | 4803 |
|---|
| Human chromosome position | 2p23 |
|---|
| LocusLink ID | 3032 |
|---|---|
| 231086 | |
| 623031 | |
| 171155 |
| Alias | ECHB |
|---|---|
| MTPB | |
| MSTP029 | |
| TP-BETA | |
| MGC87480 | |
| trifunctional enzyme subunit beta, mitochondrial | |
| beta-ketothiolase | |
| acetyl-CoA acyltransferase | |
| 2-enoyl-Coenzyme A (CoA) hydratase, beta subunit | |
| mitochondrial trifunctional enzyme, beta subunit | |
| mitochondrial trifunctional protein, beta subunit | |
| hydroxyacyl-Coenzyme A (CoA) dehydrogenase, beta subunit | |
| 3-ketoacyl-Coenzyme A (CoA) thiolase of mitochondrial trifunctional protein, beta subunit | |
| hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit | |
| 4930479F15Rik | |
| OTTMUSP00000029777 | |
| OTTMUSP00000029863 | |
| OTTMUSP00000029864 | |
| hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta aubunit | |
| OTTMUSG00000015054 | |
| 2-enoyl-coenzyme a (coa) hydratase | |
| 3-ketoacyl-Coenzyme A thiolase | |
| 4930479F15 | |
| Gm13910 | |
| Hadhb | |
| hydroxyacyl dehydrogenase, subunit B | |
| hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit | |
| hydroxyacyl-coenzyme a (coa) dehydrogenase | |
| hydroxyacyl-Coenzyme A dehydrogenase, beta subunit | |
| hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta aubunit | |
| LOC100048492 | |
| mitochondrial Trifunctional enzyme beta subunit | |
| Trifunctional enzyme beta subunit, mitochondrial | |
| trifunctional protein, beta subunit | |
| predicted gene, OTTMUSG00000015054 | |
| Trifunctional enzyme beta subunit, mitochondrial precursor | |
| RIKEN cDNA 4930479F15 gene | |
| mitochondrial Trifunctional enzyme beta subunit precursor | |
| hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit | |
| LOC623031 | |
| similar to Hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit |
| Organism | Homo sapiens |
|---|---|
| Mus musculus | |
| Rattus norvegicus |
| OMIM ID | 143450 |
|---|---|
| 609015 |
| Mouse chromosome position | 5 |
|---|---|
| 2 | |
| 5 B1 | |
| 2 E3 |
| GO ID | 0003857 |
|---|---|
| 0051287 | |
| 0003988 | |
| 0000062 | |
| 0008415 | |
| 0004300 | |
| 0016509 | |
| 0016508 | |
| 0016740 | |
| 0006635 | |
| 0006631 | |
| 0006629 | |
| 0008152 | |
| 0016507 | |
| 0005740 | |
| 0005743 | |
| 0005759 | |
| 0042645 | |
| 0005739 | |
| 0003824 | |
| 0016747 |
| Rat chromosome position | 6q14 |
|---|
| Swiss-Prot Accession | P55084 |
|---|---|
| Q99JY0 | |
| Q60587 | |
| Q3TEH9 | |
| Q8BJI5 | |
| Q8BJM0 | |
| Q8BK52 | |
| B2RB16 | |
| O14969 | |
| Q53TA6 | |
| Q96C77 | |
| Q9H3F5 | |
| Q9T2V8 |
| PIR ID | B49681 |
|---|---|
| JC2109 |
| Unigene ID | Hs.515848 |
|---|---|
| Mm.291463 | |
| Mm.389348 | |
| Rn.11253 | |
| Hs.534639 |
| KEGG ID | hsa:3032 |
|---|---|
| mmu:231086 | |
| mmu:623031 | |
| rno:171155 |
| EC Number | 2.3.1.16 |
|---|
| Swiss-Prot ID | ECHB_RAT |
|---|---|
| ECHB_MOUSE | |
| ECHB_HUMAN |
| Cell Localization | Mitochondrion matrix |
|---|---|
| Mitochondrion | |
| Cytoplasm | |
| Mitochondria |
| IPI ID | IPI00198467 |
|---|---|
| IPI00115607 | |
| IPI00022793 | |
| IPI00893108 | |
| IPI00893995 | |
| IPI00893867 | |
| IPI00893587 | |
| IPI00850941 | |
| IPI00474281 | |
| IPI00858282 |
| RGD ID | 620513 |
|---|
| MGI ID | 2136381 |
|---|---|
| 3651143 |