Any feedback?
Please rate this page
(literature.php)
(0/150)

BRENDA support

Literature summary for 1.3.1.124 extracted from

  • Miinalainen, I.; Schmitz, W.; Huotari, A.; Autio, K.; Soininen, R.; Van Themaat, E.; Baes, M.; Herzig, K.; Conzelmann, E.; Hiltunen, J.
    Mitochondrial 2,4-dienoyl-CoA reductase deficiency in mice results in severe hypoglycemia with stress intolerance and unimpaired ketogenesis (2009), PLOS Genet., 5, e1000543.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 1.3.1.124

Cloned(Commentary)

Cloned (Comment) Organism
creation of a DECR-deficient mouse line, fasted Decr-/- mice display increased serum acylcarnitines, especially decadienoylcarnitine, a product of the incomplete oxidation of linoleic acid (C18:2), urinary excretion of unsaturated dicarboxylic acids, and hepatic steatosis, wherein unsaturated fatty acids accumulate in liver triacylglycerols, metabolically challenged Decr-/- mice turn on ketogenesis, but unexpectedly develop hypoglycemia Mus musculus

Localization

Localization Comment Organism GeneOntology No. Textmining
mitochondrion
-
 Mus musculus 5739
-
peroxisome
-
 Mus musculus 5777
-

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
additional information Mus musculus 2,4-dienoyl-CoA reductase as an auxiliary enzyme in the mitochondrial beta-oxidation of unsaturated fatty acids ?
-
 ?
additional information Mus musculus DECR may also play a role in the degradation of fatty acids containing odd-numbered double bonds because the intermediate 2,5-dienoyl-CoA may be isomerized by enoyl-CoA isomerase to 3,5-dienoyl-CoA and then converted to 2,4-dienoyl-CoA by a specific delta3,5,delta2,4-dienoyl-CoA isomerase ?
-
 ?
additional information Mus musculus in eukaryotes, double bonds in even-numbered positions are reduced by an NADPH-dependent 2,4-dienoyl-CoA reductase to 3-enoyl-CoA, which is then isomerized by enoyl-CoA isomerase to trans-2-enoyl-CoA, suitable for further oxidation ?
-
 ?

Organism

Organism UniProt Comment Textmining
Mus musculus
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
heart
-
 Mus musculus
-
liver
-
 Mus musculus
-
muscle
-
 Mus musculus
-

Specific Activity [micromol/min/mg]

Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
additional information
-
 analysis of liver fatty acids after the mice are fasted for 24 h indicates that fasting has a minor effect on the lipid content of wild type liver, with an overall increase of 29% in the concentration of fatty acids, in Decr-/- mice, the overall concentration of fatty acids increases by 108% after fasting Mus musculus
additional information
-
 Decr-/- mice show decreased blood glucose and elevated non-esterified fatty acids concentrations after fasting in comparison to wild type mice Mus musculus
additional information
-
 fasting increases the total concentration of acylcarnitines by 2fold in wild type mice (567 nM), in Decr-/- mice, a markedly higher 9fold increase is observed (2150 nM) Mus musculus
additional information
-
 immunoblotting of mitochondrial extracts from liver, muscle and heart with an antibody against human DECR reveals a detectable signal from wild type mice, whereas no signal can be detected for homozygous null mutant mice Mus musculus
additional information
-
 in Decr-/- mice, the overall concentration of fatty acids increases by 108% after fasting, the most profound changes between fasted wild type and Decr-/- mice are observed for the levels of palmitoleic acid (C16:1), oleic acid, linolenic acid (C18:3) and linoleic acid, which are 2.5- to 3.8fold higher in Decr-/- mice, in comparison to the fed state, the concentrations of monounsaturated fatty acids and polyunsaturated fatty acids increase by 288% and 254%, respectively Mus musculus
additional information
-
 mitochondrial 2,4-dienoyl-CoA reductase activity in mice is indispensable for the complete oxidation of (poly)unsaturated fatty acids and for adaptation to metabolic stress Mus musculus
additional information
-
 mitochondrial 2,4-dienoyl-CoA reductase deficiency in mice results in severe hypoglycemia with stress intolerance and unimpaired ketogenesis Mus musculus
0.5
-
 reductase activity for wild-type and Decr-/- mice, the observed residual activity represents the activity of mitochondrial 2-enoyl thioester reductase, which functions in mitochondrial fatty acid synthesis and can also reduce 2,4-hexadienoyl-CoA in vitro Mus musculus
2.2
-
 reductase activity measured in liver mitochondrial extract Mus musculus
2.6
-
 reductase activity measured in muscle mitochondrial extract Mus musculus

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
additional information 2,4-dienoyl-CoA reductase as an auxiliary enzyme in the mitochondrial beta-oxidation of unsaturated fatty acids Mus musculus ?
-
 ?
additional information DECR may also play a role in the degradation of fatty acids containing odd-numbered double bonds because the intermediate 2,5-dienoyl-CoA may be isomerized by enoyl-CoA isomerase to 3,5-dienoyl-CoA and then converted to 2,4-dienoyl-CoA by a specific delta3,5,delta2,4-dienoyl-CoA isomerase Mus musculus ?
-
 ?
additional information in eukaryotes, double bonds in even-numbered positions are reduced by an NADPH-dependent 2,4-dienoyl-CoA reductase to 3-enoyl-CoA, which is then isomerized by enoyl-CoA isomerase to trans-2-enoyl-CoA, suitable for further oxidation Mus musculus ?
-
 ?

Synonyms

Synonyms Comment Organism
2,4-dienoyl coenzyme A reductase
-
 Mus musculus
2,4-dienoyl-CoA reductase
-
 Mus musculus
DECR
-
 Mus musculus

Cofactor

Cofactor Comment Organism Structure
NADPH
-
 Mus musculus