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2.3.1.199: very-long-chain 3-oxoacyl-CoA synthase

This is an abbreviated version!
For detailed information about very-long-chain 3-oxoacyl-CoA synthase, go to the full flat file.

Word Map on EC 2.3.1.199

Reaction

a very-long-chain acyl-CoA
+
malonyl-CoA
=
a very-long-chain 3-oxoacyl-CoA
 +
CO2
 +
CoA

Synonyms

3-ketoacyl CoA synthase, 3-ketoacyl-CoA synthase, acyl-CoA elongase, At1g01120, At2g26640, beta-ketoacyl CoA synthase, beta-ketoacyl-CoA synthase, Cer-zh, CER2-LIKE1, CER2-LIKE2, CER26, CER26L, CER6, CER60, condensing enzyme, Cut1, CUT1L, EC 2.3.1.119, ECERIFERUM 26, ECERIFERUM 26-like, ELO, ELo1, Elo1p, Elo2p, Elo3p, EloA, ELOVL1, ELOVL5, Elovl6, ELOVL7, FA elongase, FAE1, FAE1 beta-ketoacyl-CoA synthase, FAE1 KCS, Fatty acid elongase, fatty acid elongase 1, fatty acid elongation 1, fatty acid elongation1, KCS, Kcs1, KCS17, KCS2, KCS2/DAISY, KCS20, MALCE1, microsomal fatty acid elongase, Onion2, Os03g0220100, Os10 g0416200, seed-specific condensing enzyme, very-long-chain fatty acid condensing enzyme, very-long-chain fatty acid-condensing enzyme, VLCFA condensing enzyme, VLCFA elongase, WSL4

ECTree

     2 Transferases
         2.3 Acyltransferases
             2.3.1 Transferring groups other than aminoacyl groups
                2.3.1.199 very-long-chain 3-oxoacyl-CoA synthase

Disease

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Disease on EC 2.3.1.199 - very-long-chain 3-oxoacyl-CoA synthase

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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
Bezafibrate for x-linked adrenoleukodystrophy.
Enzymatic characterization of ELOVL1, a key enzyme in very long-chain fatty acid synthesis.
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.
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.
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.
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.
Inhibition of Secretin/Secretin Receptor Axis Ameliorates NAFLD Phenotypes.
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 molecular fingerprinting shows profound TH17 skewing and a unique barrier genomic signature.
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.
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.
Dysregulation of microRNA-125a contributes to obesity-associated insulin resistance and dysregulates lipid metabolism in mice.
Macrophage elovl6 deficiency ameliorates foam cell formation and reduces atherosclerosis in low-density lipoprotein receptor-deficient mice.
Mouse Elovl-6 promoter is an SREBP target.
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.
Multiple System Atrophy
Analysis of GWAS-linked variants in multiple system atrophy.
Investigating ELOVL7 coding variants in multiple system atrophy.
Neoplasms
Differences in elongation of very long chain fatty acids and fatty acid metabolism between triple-negative and hormone receptor-positive breast cancer.
Identification of hub genes and key pathways associated with the progression of gynecological cancer.
Novel Lipogenic Enzyme ELOVL7 Is Involved in Prostate Cancer Growth through Saturated Long-Chain Fatty Acid Metabolism.
Reactivation of androgen receptor-regulated lipid biosynthesis drives the progression of castration-resistant prostate cancer.
Signatures of Adverse Pathological Features, Androgen Insensitivity and Metastatic Potential in Prostate Cancer.
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.
Fatty acid elongase-5 (Elovl5) regulates hepatic triglyceride catabolism in obese C57BL/6J mice.
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.
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.
Transcriptomic signatures of brain regional vulnerability to Parkinson's disease.
Prostatic Neoplasms
ELOVL5 is a critical and targetable fatty acid elongase in prostate cancer.
Novel Lipogenic Enzyme ELOVL7 Is Involved in Prostate Cancer Growth through Saturated Long-Chain Fatty Acid Metabolism.
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.