Information on EC 1.1.1.211 - long-chain-3-hydroxyacyl-CoA dehydrogenase

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The expected taxonomic range for this enzyme is: Eukaryota, Bacteria

EC NUMBER
COMMENTARY hide
1.1.1.211
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RECOMMENDED NAME
GeneOntology No.
long-chain-3-hydroxyacyl-CoA dehydrogenase
REACTION
REACTION DIAGRAM
COMMENTARY hide
ORGANISM
UNIPROT
LITERATURE
a long-chain (S)-3-hydroxyacyl-CoA + NAD+ = a long-chain 3-oxoacyl-CoA + NADH + H+
show the reaction diagram
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
oxidation
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redox reaction
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reduction
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PATHWAY
BRENDA Link
KEGG Link
MetaCyc Link
(8E,10E)-dodeca-8,10-dienol biosynthesis
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Spodoptera littoralis pheromone biosynthesis
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Fatty acid elongation
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Fatty acid degradation
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Biosynthesis of unsaturated fatty acids
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Metabolic pathways
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SYSTEMATIC NAME
IUBMB Comments
long-chain-(S)-3-hydroxyacyl-CoA:NAD+ oxidoreductase
This enzyme was purified from the mitochondrial inner membrane. The enzyme has a preference for long-chain substrates, and activity with a C16 substrate was 6- to 15-fold higher than with a C4 substrate (cf. EC 1.1.1.35 3-hydroxyacyl-CoA dehydrogenase).
CAS REGISTRY NUMBER
COMMENTARY hide
84177-52-6
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GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
malfunction
metabolism
physiological function
SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
3-hydroxyoctanoyl-CoA + NAD+
3-ketooctanoyl-CoA + NADH
show the reaction diagram
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-
-
-
?
3-ketohexadecanoyl-CoA + NADH
3-hydroxyhexadecanoyl-CoA + NAD+
show the reaction diagram
3-ketohexanoyl-CoA + NADH
3-hydroxyhexanoyl-CoA + NAD+
show the reaction diagram
3-oxodecanoyl-CoA + NADH
3-hydroxydecanoyl-CoA + NAD+
show the reaction diagram
a long-chain (S)-3-hydroxyacyl-CoA + NAD+
a long-chain 3-oxoacyl-CoA + NADH + H+
show the reaction diagram
acetoacetyl-CoA + NADH
3-hydroxybutyryl-CoA + NAD+
show the reaction diagram
additional information
?
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NATURAL SUBSTRATES
NATURAL PRODUCTS
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
a long-chain (S)-3-hydroxyacyl-CoA + NAD+
a long-chain 3-oxoacyl-CoA + NADH + H+
show the reaction diagram
additional information
?
-
INHIBITORS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
defatted serum albumin
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ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
defatted serum albumin
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KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
0.06
NAD+
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0.05
NADH
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SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
3.2 - 7.4
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3-hydroxyoctanoyl-CoA oxidation
15 - 35
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3-ketohexadecanoyl-CoA reduction
16.3
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3-ketohexadecanoyl-CoA reduction
30.4
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3-ketohexadecanoyl-CoA reduction
additional information
pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
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fetal, strong expression
Manually annotated by BRENDA team
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developing, fetal, strong expression
Manually annotated by BRENDA team
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Manually annotated by BRENDA team
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fetal, strong expression
Manually annotated by BRENDA team
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adult and fetal
Manually annotated by BRENDA team
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neural, developing
Manually annotated by BRENDA team
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retinal pigment epithelial monolayers are constructed by human induced pluripotent stem cell retina technology
Manually annotated by BRENDA team
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Manually annotated by BRENDA team
additional information
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long-chain fatty acid oxidation during fetal and early human development, overview
Manually annotated by BRENDA team
MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
47000
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alpha2,beta2, 1 * 71000 + 1 * 47000, SDS-PAGE
51000
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alpha4beta4, 4 * 79000 + 4 * 51000, SDS-PAGE
71000
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alpha2,beta2, 1 * 71000 + 1 * 47000, SDS-PAGE
79000
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alpha4beta4, 4 * 79000 + 4 * 51000, SDS-PAGE
186000
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liver, gel filtration
230000
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liver, gel filtration
460000
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gel filtration
SUBUNITS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
octamer
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alpha4beta4, 4 * 79000 + 4 * 51000, SDS-PAGE
tetramer
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alpha2,beta2, 1 * 71000 + 1 * 47000, SDS-PAGE
additional information
Purification/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
trifunctional enzyme: 2-enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase/3-oxoacyl-CoA thiolase
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Cloned/COMMENTARY
ORGANISM
UNIPROT
LITERATURE
DNA and amino acid sequence determination and analysis of genomic DNA from a women who survived acute fatty liver in pregnancy, the women does not carry the G1528C polymorphism
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gene HADHA
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into XbaI- and HindIII-digested pK18mobsacB, resulting in plasmid pKXZ01
EXPRESSION
ORGANISM
UNIPROT
LITERATURE
nickel strongly represses mitochondrial fatty acid oxidation, the pathway by which fatty acids are catabolized for energy, in both primary human lung fibroblasts and mouse embryonic fibroblasts
the enzyme is expressed and distributed widely in maternal and fetal tissues during the middle trimester
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ENGINEERING
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
G1528C
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a common polymorphism, which is probably not involved in enzyme deficiency in acute fatty liver in pregnancy
Q358K
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heterozygous mutation in exon 11, 1072C/A, a naturally mutation occuring in the LCHAD coding region, HADHA, causing growth-restriction in a 25-4/7 week gestation female infant
additional information
APPLICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
degradation
diagnostics
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3-hydroxypalmitoleoyl-carnitine and other hydroxylated long chain acylcarnitines are markers of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD) and/or trifunctional protein (TFP) deficiency, while heptadecanoylcarnitine is a biomarker specific for propionic acidemia (PA) or methylmalonic acidemia (MMA) patients able to detect propionate disorders during expanded newborn screening
medicine