DECR1 gene

2,4-dienoyl-CoA reductase 1, mitochondrial

The information on this page was automatically extracted from online scientific databases.

From NCBI Gene:

This gene encodes an accessory enzyme which participates in the beta-oxidation and metabolism of unsaturated fatty enoyl-CoA esters. [provided by RefSeq, Jul 2008]

From UniProt:

Auxiliary enzyme of beta-oxidation. It participates in the metabolism of unsaturated fatty enoyl-CoA esters having double bonds in both even- and odd-numbered positions. Catalyzes the NADP-dependent reduction of 2,4-dienoyl-CoA to yield trans-3-enoyl-CoA.

From UniProt:

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. [MIM:616034]

Cytogenetic Location: 8q21.3, which is the long (q) arm of chromosome 8 at position 21.3

Molecular Location: base pairs 90,001,337 to 90,052,004 on chromosome 8 (Homo sapiens Annotation Release 108, GRCh38.p7) (NCBI)

Cytogenetic Location: 8q21.3, which is the long (q) arm of chromosome 8 at position 21.3
  • DECR
  • NADPH
  • SDR18C1