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Information on EC 2.3.1.199 - very-long-chain 3-oxoacyl-CoA synthase
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EC Tree
2.3.1.199 very-long-chain 3-oxoacyl-CoA synthaseIUBMB Comments
This is the first component of the elongase, a microsomal protein complex responsible for extending palmitoyl-CoA and stearoyl-CoA (and modified forms thereof) to very-long-chain acyl CoAs. Multiple forms exist with differing preferences for the substrate, and thus the specific form expressed determines the local composition of very-long-chain fatty acids [6,7]. For example, the FAE1 form from the plant Arabidopsis thaliana accepts only 16 and 18 carbon substrates, with oleoyl-CoA (18:1) being the preferred substrate , while CER6 from the same plant prefers substrates with chain length of C22 to C32 [4,8]. cf. EC 1.1.1.330, very-long-chain 3-oxoacyl-CoA reductase, EC 4.2.1.134, very-long-chain (3R)-3-hydroxyacyl-[acyl-carrier protein] dehydratase, and EC 1.3.1.93, very-long-chain enoyl-CoA reductase
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Word Map
- 2.3.1.199
-
vlcfas
-
polyunsaturated
- acyl-coas
-
desaturase
-
elovls
-
monounsaturated
- cuticular
- ceramide
-
erucic
-
pufas
- sphingolipids
- stearoyl-coa
-
oilseed
-
epicuticular
- adrenoleukodystrophy
-
eicosenoic
-
delta6
-
docosapentaenoic
-
napins
-
vlc-pufas
- leek
- suberins
- medicine
The expected taxonomic range for this enzyme is: Eukaryota, Bacteria
Reaction Schemes
Synonyms
fatty acid elongase, elovl1, elovl7, 3-ketoacyl-coa synthase, fatty acid elongase 1, acyl-coa elongase, beta-ketoacyl-coa synthase, fa elongase, fae1 kcs, fatty acid elongation1, 
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SYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
3-ketoacyl-CoA synthase
acyl-CoA elongase
beta-ketoacyl-CoA synthase
CER6
CER60
Cut1
EC 2.3.1.119
-
-
formerly, part transferred
-
ELo1
Elo1p
EloA
FAE1
Fatty acid elongase
fatty acid elongase 1
KCS
Kcs1
KCS20
MALCE1
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REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT
LITERATURE
a very-long-chain acyl-CoA + malonyl-CoA = a very-long-chain 3-oxoacyl-CoA + CO2 + CoA
-
-
-
-
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PATHWAY SOURCE
PATHWAYS
KEGG
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SYSTEMATIC NAME
IUBMB Comments
malonyl-CoA:very-long-chain acyl-CoA malonyltransferase (decarboxylating and thioester-hydrolysing)
This is the first component of the elongase, a microsomal protein complex responsible for extending palmitoyl-CoA and stearoyl-CoA (and modified forms thereof) to very-long-chain acyl CoAs. Multiple forms exist with differing preferences for the substrate, and thus the specific form expressed determines the local composition of very-long-chain fatty acids [6,7]. For example, the FAE1 form from the plant Arabidopsis thaliana accepts only 16 and 18 carbon substrates, with oleoyl-CoA (18:1) being the preferred substrate [5], while CER6 from the same plant prefers substrates with chain length of C22 to C32 [4,8]. cf. EC 1.1.1.330, very-long-chain 3-oxoacyl-CoA reductase, EC 4.2.1.134, very-long-chain (3R)-3-hydroxyacyl-[acyl-carrier protein] dehydratase, and EC 1.3.1.93, very-long-chain enoyl-CoA reductase
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CAS REGISTRY NUMBER
COMMENTARY
88414-92-0
-
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SUBSTRATE
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
(9Z,12Z)-octadec-9,12-dienoyl-CoA + malonyl-CoA
(11Z,14Z)-3-oxoeicosa-11,14-dienoyl-CoA + CO2 + coenzyme A
(9Z,12Z,15Z)-octadeca-9,12,15-trienoyl-CoA + malonyl-CoA
(11Z,14Z,17Z)-3-oxoeicosa-11,14,17-trienoyl-CoA + CO2 + coenzyme A
a very-long-chain acyl-CoA + malonyl-CoA
a very-long-chain 3-oxoacyl-CoA + CO2 + coenzyme A
-
-
-
?
oleoyl-CoA + malonyl-CoA
?
-
in contrast to stearic acid-CoA, oleic acid-CoA serves as a less suitable substrate for elongation
-
-
?
oleoyl-CoA + malonyl-CoA
CoA + 3-oxo-eicosenoyl-CoA + 3-oxo-erucoyl-CoA + CO2
-
preferred substrate
3-oxo-eicosenoyl-CoA is the major product
-
?
stearoyl-CoA + malonyl-CoA
CoA + 3-oxo-eicosanoyl-CoA + CO2
-
-
major product
-
?
(9Z)-hexadec-9-enoyl-CoA + malonyl-CoA
(11Z)-3-oxooctadec-11-enoyl-CoA + CO2 + coenzyme A
-
-
-
ir
(9Z)-hexadec-9-enoyl-CoA + malonyl-CoA
(11Z)-3-oxooctadec-11-enoyl-CoA + CO2 + coenzyme A
-
-
-
ir
(9Z,12Z)-octadec-9,12-dienoyl-CoA + malonyl-CoA
(11Z,14Z)-3-oxoeicosa-11,14-dienoyl-CoA + CO2 + coenzyme A
-
-
-
ir
(9Z,12Z)-octadec-9,12-dienoyl-CoA + malonyl-CoA
(11Z,14Z)-3-oxoeicosa-11,14-dienoyl-CoA + CO2 + coenzyme A
-
-
-
ir
(9Z,12Z,15Z)-octadeca-9,12,15-trienoyl-CoA + malonyl-CoA
(11Z,14Z,17Z)-3-oxoeicosa-11,14,17-trienoyl-CoA + CO2 + coenzyme A
-
-
-
?
(9Z,12Z,15Z)-octadeca-9,12,15-trienoyl-CoA + malonyl-CoA
(11Z,14Z,17Z)-3-oxoeicosa-11,14,17-trienoyl-CoA + CO2 + coenzyme A
-
-
-
?
a very-long-chain acyl-CoA + malonyl-CoA
a very-long-chain 3-oxoacyl-CoA + CO2 + CoA
-
-
-
-
?
a very-long-chain acyl-CoA + malonyl-CoA
a very-long-chain 3-oxoacyl-CoA + CO2 + CoA
-
-
-
?
arachidoyl-CoA + malonyl-CoA
?
-
25% activity compared to oleoyl-CoA
-
-
?
stearoyl-CoA + malonyl-CoA
?
-
best substrate for At2g26640 and FAE1
-
-
?
very-long-chain acyl-CoA + malonyl-CoA
CoA + very-long-chain 3-oxoacyl-CoA + CO2
-
-
-
-
?
very-long-chain acyl-CoA + malonyl-CoA
CoA + very-long-chain 3-oxoacyl-CoA + CO2
-
-
-
-
?
very-long-chain acyl-CoA + malonyl-CoA
CoA + very-long-chain 3-oxoacyl-CoA + CO2
-
-
-
-
?
very-long-chain acyl-CoA + malonyl-CoA
CoA + very-long-chain 3-oxoacyl-CoA + CO2
-
-
-
-
?
additional information
?
-
-
At1g01120 (KCS1) and At2g26640 have broad substrate specificities when assayed with saturated and monounsaturated C16 to C24 acyl-CoAs while At4g34510 is specific for saturated fatty acyl-CoA substrates. KCS1 is most active with palmitic acid, palmitoleic acid, stearic acid, and arachidic acid but has very little activity with oleic acid and acyl chains longer than C20. At4g34510 is specific for saturated acyl-CoAs up to C22. FAE1 uses palmitic acid, palmitoleic acid, stearic acid, oleic acid, arachidic acid, 20:1, behenic acid, erucic acid, and lignoceric acid as substratesis and is specific for acyl-CoAs of 16 and 18 carbons in length
-
-
?
additional information
?
-
-
the enzyme shows highest activity towards saturated and monounsaturated C16 and C18. In the absence of an acyl-CoA substrate, the enzyme is unable to carry out decarboxylation of malonyl-CoA
-
-
?
additional information
?
-
-
the enzyme shows no activity with polyunsaturated linoleic acid and alpha-linolenic acid and little or no activity with acyl-CoAs having 22 carbons or longer in chain length
-
-
?
additional information
?
-
the effect of CER2-LIKE1 and CER2-LIKE2 proteins on fatty acid elongation is limited to specific condensing enzyme partners, and CER2-LIKE proteins either have their own unique substrate specificity, or a unique effect on the substrate specificity of the condensing enzyme with which they function
-
-
?
additional information
?
-
the effect of CER2-LIKE1 and CER2-LIKE2 proteins on fatty acid elongation is limited to specific condensing enzyme partners, and CER2-LIKE proteins either have their own unique substrate specificity, or a unique effect on the substrate specificity of the condensing enzyme with which they function
-
-
?
additional information
?
-
almost no activity with palmitic acid and 17:1DELTA9
-
-
?
additional information
?
-
-
almost no activity with palmitic acid and 17:1DELTA9
-
-
?
additional information
?
-
almost no activity with palmitic acid and 17:1DELTA9
-
-
?
additional information
?
-
the enzyme is able to elongate the C16-acyl-CoA up to C26-acyl-CoA
-
-
?
additional information
?
-
-
the enzyme is able to elongate the C16-acyl-CoA up to C26-acyl-CoA
-
-
?
additional information
?
-
no activity with saturated and unsaturated fatty acids longer than C16
-
-
?
additional information
?
-
-
the enzyme is specific for acyl chain lengths between 16 and 20 carbons
-
-
-
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NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
a very-long-chain acyl-CoA + malonyl-CoA
a very-long-chain 3-oxoacyl-CoA + CO2 + coenzyme A
-
-
-
?
a very-long-chain acyl-CoA + malonyl-CoA
a very-long-chain 3-oxoacyl-CoA + CO2 + CoA
-
-
-
-
?
a very-long-chain acyl-CoA + malonyl-CoA
a very-long-chain 3-oxoacyl-CoA + CO2 + CoA
-
-
-
?
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COFACTOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
additional information
-
CoA, NADPH and ATP have no effect on the condensation activity of the recombinant enzyme
-
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METALS and IONS
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
mefluidide
-
selective inhibitor of KCS1, CER6, and CER60 enzyme activities in vivo. FAE1 and KCS2 are inhibited by mefluidide only slightly. At 0.1 mM mefluidide, isozymes FAE1, KCS1, KCS2, KCS20, CER60, and CER6 are inhibited by 5.1%, 91%, 0.3%, 26.9%, 71.8%, 51.2% respectively
metazachlor
-
selective inhibitor of KCS1, CER6, and CER60 enzyme activities in vivo. Strong micromolar inhibitor of FAE1 and KCS2. At 0.1 mM metazachlor, isozymes FAE1, KCS1, KCS2, KCS20, CER60, CER6, and KCS17 are inhibited by 95.7%, 93%, 82.4%, 97.5%, 100%, 100%, and 100%, respectively
perfluidone
-
selective inhibitor of KCS1, CER6, and CER60 enzyme activities in vivo. Strong micromolar inhibitor of FAE1 and KCS2
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ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
NADPH
ELOVL6 activity in membrane fractions is enhanced in the presence of NADPH
additional information
the 3-ketoacyl-CoA reductase, KAR, the enzyme catalyzing the next step in metabolism after fatty acid elongase ELOVL6, largely stimulates ELOVL6 activity. Activity of purified enzyme ELOVL6 is enhanced by about 3fold in the presence of 3-ketoacyl-CoA reductase, KAR. This effect is KAR enzyme activity-independent, since it is observed in the absence of NADPH and in the poorly active or inactive KAR mutants, overview. ELOVL6 enzyme activity is also enhanced in a KAR enzyme activity-dependent manner
-
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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
Acanthosis Nigricans
De novo mutation in ELOVL1 causes ichthyosis, acanthosis nigricans, hypomyelination, spastic paraplegia, high frequency deafness and optic atrophy.
Adrenoleukodystrophy
Enzymatic characterization of ELOVL1, a key enzyme in very long-chain fatty acid synthesis.
Adrenoleukodystrophy
The role of ELOVL1 in very long-chain fatty acid homeostasis and X-linked adrenoleukodystrophy.
Breast Neoplasms
Estrogen Enhances the Expression of the Polyunsaturated Fatty Acid Elongase Elovl2 via ER? in Breast Cancer Cells.
Breast Neoplasms
Novel theranostic opportunities offered by characterization of altered membrane lipid metabolism in breast cancer progression.
Corneal Opacity
Very long-chain tear film lipids produced by fatty acid elongase ELOVL1 prevent dry eye disease in mice.
Deafness
De novo mutation in ELOVL1 causes ichthyosis, acanthosis nigricans, hypomyelination, spastic paraplegia, high frequency deafness and optic atrophy.
Deafness
Reduced chain length in myelin sphingolipids and poorer motor coordination in mice deficient in the fatty acid elongase Elovl1.
Demyelinating Diseases
Dicer ablation in oligodendrocytes provokes neuronal impairment in mice.
Dermatitis
IFN-? Reduces Epidermal Barrier Function by Affecting Fatty Acid Composition of Ceramide in a Mouse Atopic Dermatitis Model.
Dry Eye Syndromes
Improvement of Evaporative Dry Eye With Meibomian Gland Dysfunction in Model Mice by Treatment With Ophthalmic Solution Containing Mineral Oil.
Dry Eye Syndromes
Very long-chain tear film lipids produced by fatty acid elongase ELOVL1 prevent dry eye disease in mice.
Fatty Liver
Fatty acid elongase-5 (Elovl5) regulates hepatic triglyceride catabolism in obese C57BL/6J mice.
Hyperglycemia
Disrupted sphingolipid metabolism following acute clozapine and olanzapine administration.
Hyperkeratosis, Epidermolytic
Ichthyosis molecular fingerprinting shows profound TH17 skewing and a unique barrier genomic signature.
Ichthyosis
De novo mutation in ELOVL1 causes ichthyosis, acanthosis nigricans, hypomyelination, spastic paraplegia, high frequency deafness and optic atrophy.
Ichthyosis
Ichthyosis molecular fingerprinting shows profound TH17 skewing and a unique barrier genomic signature.
Ichthyosis
Reduced chain length in myelin sphingolipids and poorer motor coordination in mice deficient in the fatty acid elongase Elovl1.
Infections
Fatty Acid elongase 7 catalyzes lipidome remodeling essential for human cytomegalovirus replication.
Infections
Weighted Gene Co-Expression Network Analysis Identifies Key Modules and Hub Genes Associated with Mycobacterial Infection of Human Macrophages.
Insulin Resistance
Crucial role of a long-chain fatty acid elongase, Elovl6, in obesity-induced insulin resistance.
Insulin Resistance
Dysregulation of microRNA-125a contributes to obesity-associated insulin resistance and dysregulates lipid metabolism in mice.
Insulin Resistance
Macrophage elovl6 deficiency ameliorates foam cell formation and reduces atherosclerosis in low-density lipoprotein receptor-deficient mice.
Insulin Resistance
Role of fatty acid elongase Elovl6 in the regulation of energy metabolism and pathophysiological significance in diabetes.
Macular Degeneration
Loss of ER retention and sequestration of the wild-type ELOVL4 by Stargardt disease dominant negative mutants.
Multiple Myeloma
The fatty acid elongase ELOVL6 regulates bortezomib resistance in multiple myeloma.
Neoplasms
Differences in elongation of very long chain fatty acids and fatty acid metabolism between triple-negative and hormone receptor-positive breast cancer.
Neoplasms
Identification of hub genes and key pathways associated with the progression of gynecological cancer.
Neoplasms
Novel Lipogenic Enzyme ELOVL7 Is Involved in Prostate Cancer Growth through Saturated Long-Chain Fatty Acid Metabolism.
Neoplasms
Reactivation of androgen receptor-regulated lipid biosynthesis drives the progression of castration-resistant prostate cancer.
Neoplasms
Signatures of Adverse Pathological Features, Androgen Insensitivity and Metastatic Potential in Prostate Cancer.
Neoplasms
Systematic integration of molecular profiles identifies miR-22 as a regulator of lipid and folate metabolism in breast cancer cells.
Neurocutaneous Syndromes
Reduced chain length in myelin sphingolipids and poorer motor coordination in mice deficient in the fatty acid elongase Elovl1.
Non-alcoholic Fatty Liver Disease
The IGF2 mRNA binding protein p62/IGF2BP2-2 induces fatty acid elongation as a critical feature of steatosis.
Obesity
Ablation of the very-long-chain fatty acid elongase ELOVL3 in mice leads to constrained lipid storage and resistance to diet-induced obesity.
Obesity
Fatty acid elongase-5 (Elovl5) regulates hepatic triglyceride catabolism in obese C57BL/6J mice.
Obesity
Regulation of hepatic fatty acid elongase and desaturase expression in diabetes and obesity.
Optic Atrophy
De novo mutation in ELOVL1 causes ichthyosis, acanthosis nigricans, hypomyelination, spastic paraplegia, high frequency deafness and optic atrophy.
Paraplegia
De novo mutation in ELOVL1 causes ichthyosis, acanthosis nigricans, hypomyelination, spastic paraplegia, high frequency deafness and optic atrophy.
Paraplegia
Reduced chain length in myelin sphingolipids and poorer motor coordination in mice deficient in the fatty acid elongase Elovl1.
Parkinson Disease
Association of GALC, ZNF184, IL1R2 and ELOVL7 With Parkinson's Disease in Southern Chinese.
Parkinson Disease
Transcriptomic signatures of brain regional vulnerability to Parkinson's disease.
Prostatic Neoplasms
ELOVL5 is a critical and targetable fatty acid elongase in prostate cancer.
Prostatic Neoplasms
Novel Lipogenic Enzyme ELOVL7 Is Involved in Prostate Cancer Growth through Saturated Long-Chain Fatty Acid Metabolism.
Prostatic Neoplasms
The chromatin remodeling protein BRG1 links ELOVL3 trans-activation to prostate cancer metastasis.
Protein Deficiency
Enzymatic characterization of ELOVL1, a key enzyme in very long-chain fatty acid synthesis.
Rubella
Regulation of FATTY ACID ELONGATION1 expression and production in Brassica oleracea and Capsella rubella.
Starvation
Identification of a ?6 fatty acid elongase gene for arachidonic acid biosynthesis localized to the endoplasmic reticulum in the green microalga Myrmecia incisa Reisigl.
very-long-chain 3-oxoacyl-coa synthase deficiency
Reduced chain length in myelin sphingolipids and poorer motor coordination in mice deficient in the fatty acid elongase Elovl1.
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KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
0.83
decanoyl-CoA
-
in 10 mM Tris/maleic acid pH 6.7, 0.65 M mannitol and 0.36 mM EGTA, at 30°C
0.05
lauroyl-CoA
-
in 10 mM Tris/maleic acid pH 6.7, 0.65 M mannitol and 0.36 mM EGTA, at 30°C
0.4
myristoyl-CoA
-
in 10 mM Tris/maleic acid pH 6.7, 0.65 M mannitol and 0.36 mM EGTA, at 30°C
0.33
octanoyl-CoA
-
in 10 mM Tris/maleic acid pH 6.7, 0.65 M mannitol and 0.36 mM EGTA, at 30°C
0.13
palmitoyl-CoA
-
in 10 mM Tris/maleic acid pH 6.7, 0.65 M mannitol and 0.36 mM EGTA, at 30°C
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IC50 VALUE [mM]
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
0.000032
mefluidide
Arabidopsis thaliana
-
isozyme CER6, pH and temperature not specified in the publication
0.00199
mefluidide
Arabidopsis thaliana
-
isozyme FAE1, pH and temperature not specified in the publication
0.0076
metazachlor
Arabidopsis thaliana
-
isozyme FAE1, pH and temperature not specified in the publication
0.114
metazachlor
Arabidopsis thaliana
-
isozyme CER6, pH and temperature not specified in the publication
0.00021
perfluidone
Arabidopsis thaliana
-
isozyme CER6, pH and temperature not specified in the publication
0.5
perfluidone
Arabidopsis thaliana
-
IC50 above 0.5 mM, isozyme FAE1, pH and temperature not specified in the publication
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pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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TEMPERATURE OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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pI VALUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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ORGANISM
COMMENTARY
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
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SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
SOURCE