Entity Details
| Primary name |
DECR_HUMAN |
| Entity type |
UniProt |
| Source |
Source Link |
Details
| Accession | Q16698 |
| EntryName | DECR_HUMAN |
| FullName | 2,4-dienoyl-CoA reductase [(3E)-enoyl-CoA-producing], mitochondrial |
| TaxID | 9606 |
| Evidence | evidence at protein level |
| Length | 335 |
| SequenceStatus | complete |
| DateCreated | 1998-12-15 |
| DateModified | 2021-06-02 |
Subcellular Location
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| Subcellular Location |
| Mitochondrion |
Domains
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| Domain | Name | Category | Type |
| IPR002347 | Short-chain dehydrogenase/reductase SDR | Family | Family |
| IPR036291 | NAD(P)-binding domain superfamily | Family | Homologous superfamily |
Diseases
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| Disease ID | Source | Name | Description |
| 616034 | OMIM | 2,4-dienoyl-CoA reductase deficiency (DECRD) | A rare, autosomal recessive, inborn error of polyunsaturated fatty acids and lysine metabolism, resulting in mitochondrial dysfunction. Affected individuals have a severe encephalopathy with neurologic and metabolic abnormalities beginning in early infancy. Laboratory studies show increased C10:2 carnitine levels and hyperlysinemia. The protein represented in this entry is involved in disease pathogenesis. A selective decrease in mitochondrial NADP(H) levels due to NADK2 mutations causes a deficiency of NADPH-dependent mitochondrial enzymes, such as DECR1 and AASS. |
Drugs
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| Drug | Name | Source | Type |
| DB03461 | Nicotinamide adenine dinucleotide phosphate | Drugbank | small molecule |
| DB08605 | 6-METHYL-2(PROPANE-1-SULFONYL)-2H-THIENO[3,2-D][1,2,3]DIAZABORININ-1-OL | Drugbank | small molecule |
Interactions
3 interactions