| URN | urn:agi-llid:3027 |
|---|---|
| Connectivity | 19 |
| Name | Hadh |
| Description | hydroxyacyl-Coenzyme A dehydrogenase |
| Notes | This gene is a member of the 3-hydroxyacyl-CoA dehydrogenase gene family. The encoded protein functions in the mitochondrial matrix to catalyze the oxidation of straight-chain 3-hydroxyacyl-CoAs as part of the beta-oxidation pathway. Its enzymatic activity is highest with medium-chain-length fatty acids. Mutations in this gene cause one form of familial hyperinsulinemic hypoglycemia. The human genome contains a related pseudogene. |
| Pathway | fatty acid beta degradation pathway |
|---|
| GO Molecular Function | catalytic activity |
|---|---|
| oxidoreductase activity | |
| coenzyme binding | |
| NAD binding | |
| 3-hydroxyacyl-CoA dehydrogenase activity |
| GO Cellular Component | cytoplasm |
|---|---|
| mitochondrion | |
| mitochondrial inner membrane | |
| mitochondrial matrix |
| GO Biological Process | metabolic process |
|---|---|
| oxidation reduction | |
| response to activity | |
| negative regulation of insulin secretion | |
| fatty acid metabolic process | |
| fatty acid beta-oxidation | |
| response to drug | |
| response to hormone stimulus | |
| response to insulin stimulus | |
| 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 | |
| response to activity | |
| negative regulation of insulin secretion | |
| fatty acid metabolic process | |
| fatty acid beta-oxidation | |
| response to drug | |
| response to hormone stimulus | |
| response to insulin stimulus | |
| lipid metabolic process | |
| oxidoreductase activity | |
| coenzyme binding | |
| NAD binding | |
| 3-hydroxyacyl-CoA dehydrogenase activity | |
| cytoplasm | |
| mitochondrion | |
| mitochondrial inner membrane | |
| mitochondrial matrix |
| MedScan ID | 3027 |
|---|
| Hugo ID | 4799 |
|---|
| Human chromosome position | 4q22-q26 |
|---|
| LocusLink ID | 3027 |
|---|---|
| 3033 | |
| 15107 | |
| 113965 |
| Alias | HAD |
|---|---|
| HCDH | |
| HHF4 | |
| HADH1 | |
| SCHAD | |
| HADHSC | |
| MSCHAD | |
| MGC8392 | |
| hydroxyacyl-coenzyme A dehydrogenase, mitochondrial | |
| OTTHUMP00000162626 | |
| short-chain 3-hydroxyacyl-CoA dehydrogenase | |
| L-3-hydroxyacyl-Coenzyme A dehydrogenase, short chain | |
| medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase | |
| AA409008 | |
| AU019341 | |
| AW742602 | |
| hydroxylacyl-Coenzyme A dehydrogenase | |
| short chain 3-hydroxyacyl-CoA dehydrogenase | |
| hydroxylacyl-Coenzyme A dehydrogenase-dehydrogenase | |
| medium and short chain L-3-hydroxyacyl-coenzyme A dehydrogenase | |
| hydroxylacyl-Coenzyme A dehydrogenase short chain | |
| hydroxylacyl-Coenzyme A dehydrogenase, short chain | |
| HADH | |
| hydroxyacyl-CoA dehydrogenase | |
| hydroxyacyl-Coenzyme A dehydrogenase | |
| L-3-hydroxyacyl-CoA dehydrogenase, short chain | |
| L-3-hydroxyacyl-Coenzyme A dehydrogenase | |
| LOC360353 | |
| M/SCHAD | |
| mitochondrial Short chain 3-hydroxyacyl-CoA dehydrogenase | |
| Short chain 3-hydroxyacyl-CoA dehydrogenase, mitochondrial | |
| mitochondrial Short chain 3-hydroxyacyl-CoA dehydrogenase precursor | |
| Short chain 3-hydroxyacyl-CoA dehydrogenase, mitochondrial precursor | |
| expressed sequence AA409008 | |
| medium and short chain |
| Organism | Homo sapiens |
|---|---|
| Mus musculus | |
| Rattus norvegicus |
| OMIM ID | 601609 |
|---|---|
| 231530 | |
| 609975 |
| Mouse chromosome position | 3 |
|---|---|
| 3 G3 |
| GO ID | 0003857 |
|---|---|
| 0050662 | |
| 0016491 | |
| 0006631 | |
| 0006629 | |
| 0055114 | |
| 0005737 | |
| 0005759 | |
| 0005739 | |
| 0003824 | |
| 0006635 | |
| 0008152 | |
| 0046676 | |
| 0014823 | |
| 0042493 | |
| 0032868 | |
| 0005743 | |
| 0070403 | |
| 0009725 | |
| 0005488 | |
| 0005515 |
| Rat chromosome position | 2q43 |
|---|
| Swiss-Prot Accession | Q16836 |
|---|---|
| Q4W5B4 | |
| Q61425 | |
| Q9WVK7 | |
| Q3TF75 | |
| Q3THK8 | |
| Q3UFI0 | |
| Q8K149 | |
| Q925U9 | |
| O00324 | |
| O00397 | |
| O00753 |
| PIR ID | S74114 |
|---|---|
| JC4210 | |
| JC4879 |
| Unigene ID | Hs.438289 |
|---|---|
| Mm.260164 | |
| Rn.92789 |
| KEGG ID | hsa:3033 |
|---|---|
| mmu:15107 | |
| rno:113965 |
| EC Number | 1.1.1.35 |
|---|
| Swiss-Prot ID | HCDH_RAT |
|---|---|
| HCDH_MOUSE | |
| HCDH_HUMAN |
| Cell Localization | Mitochondrion matrix |
|---|---|
| Cytoplasm | |
| Mitochondria |
| IPI ID | IPI00205157 |
|---|---|
| IPI00121105 | |
| IPI00294398 | |
| IPI00298406 |
| Homologene ID | 55888 |
|---|
| RGD ID | 69321 |
|---|
| MGI ID | 96009 |
|---|