2,4 Dienoyl-CoA reductase

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Oxford Dictionary of Biochemistry:

2,4-dienoyl-CoA reductase

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(NADPH)

EC 1.3.1.34; systematic name: trans-2,3-didehydroacyl-CoA:NADP+ 4-oxidoreductase; other name: 4-enoyl-CoA reductase (NADPH). An enzyme of the pathway for oxidation of unsaturated fatty acids that catalyses the reduction by NADPH of trans,trans-2,3,4,5-tetradehydroacyl-CoA to trans-2,3-didehydroacyl- CoA, a normal substrate of β oxidation. For the bacterial enzyme, this is a direct product of the enzyme, but for the mammalian enzyme the product is trans-3,4-didehydroacyl- CoA, which must undergo isomerization to trans-2,3-didehydroacyl- CoA. See also unsaturated fatty acid oxidation.

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Wikipedia on Answers.com:

2,4 Dienoyl-CoA reductase

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2,4-dienoyl CoA reductase 1, mitochondrial
DHFR.png
Crystallographic structure of a tetramer of the ternary complex of DECR1, hexanoyl-coenzyme A, and NADP.[1]
Identifiers
Symbol DECR1
Alt. symbols DECR
Entrez 1666
HUGO 2753
OMIM 222745
PDB 1w6u
RefSeq NM_001359
UniProt Q16698
Other data
EC number 1.3.1.34
Locus Chr. 8 q21.3

2,4 Dienoyl-CoA reductase also known as DECR1 is a protein which in humans is encoded by the DECR1 gene which resides on chromosome 8.[2][3][4]

This gene encodes an accessory enzyme which participates in the beta oxidation and metabolism of unsaturated fatty enoyl-CoA esters.

Contents

Pathology

Mutations in the DECR1 gene may result in 2,4 Dienoyl-CoA reductase deficiency.[5]

See also

References

  1. ^ PDB 1w6u; Alphey MS, Yu W, Byres E, Li D, Hunter WN (January 2005). "Structure and reactivity of human mitochondrial 2,4-dienoyl-CoA reductase: enzyme-ligand interactions in a distinctive short-chain reductase active site". J. Biol. Chem. 280 (4): 3068–77. doi:10.1074/jbc.M411069200. PMID 15531764. 
  2. ^ "Entrez Gene: 2,4-dienoyl CoA reductase 1, mitochondrial". http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=1666. 
  3. ^ Koivuranta KT, Hakkola EH, Hiltunen JK (December 1994). "Isolation and characterization of cDNA for human 120 kDa mitochondrial 2,4-dienoyl-coenzyme A reductase". Biochem. J. 304 (3): 787–92. PMC 1137403. PMID 7818482. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1137403. 
  4. ^ Helander HM, Koivuranta KT, Horelli-Kuitunen N, Palvimo JJ, Palotie A, Hiltunen JK (November 1997). "Molecular cloning and characterization of the human mitochondrial 2,4-dienoyl-CoA reductase gene (DECR)". Genomics 46 (1): 112–9. doi:10.1006/geno.1997.5004. PMID 9403065. 
  5. ^ Roe CR, Millington DS, Norwood DL, Kodo N, Sprecher H, Mohammed BS, Nada M, Schulz H, McVie R (May 1990). "2,4-Dienoyl-coenzyme A reductase deficiency: a possible new disorder of fatty acid oxidation". J. Clin. Invest. 85 (5): 1703–7. doi:10.1172/JCI114624. PMC 296625. PMID 2332510. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=296625. 

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