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Information on EC 1.3.8.9 - very-long-chain acyl-CoA dehydrogenase
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1.3.8.9 very-long-chain acyl-CoA dehydrogenaseIUBMB Comments
Contains FAD as prosthetic group. One of several enzymes that catalyse the first step in fatty acids beta-oxidation. The enzyme is most active toward long-chain acyl-CoAs such as C14, C16 and C18, but is also active with very-long-chain acyl-CoAs up to 24 carbons. It shows no activity for substrates of less than 12 carbons. Its specific activity towards palmitoyl-CoA is more than 10-fold that of the long-chain acyl-CoA dehydrogenase . cf. EC 1.3.8.1, short-chain acyl-CoA dehydrogenase, EC 1.3.8.7, medium-chain acyl-CoA dehydrogenase, and EC 1.3.8.8, long-chain acyl-CoA dehydrogenase.
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Word Map
- 1.3.8.9
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beta-oxidation
- acylcarnitine
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medium-chain
- carnitine
- hypoglycemia
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vlcadd
- rhabdomyolysis
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vlcad-deficient
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trifunctional
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hypoketotic
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myopathic
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faods
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dehydrogenase-deficient
- 3-hydroxyacyl-coa
- myoglobinuria
- hadha
The enzyme appears in viruses and cellular organisms
Reaction Schemes
Synonyms
acadvl, 
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SYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
ACADVL
very long chain acyl-CoA dehydrogenase
very long-chain acyl-CoA dehydrogenase
very-long-chain acyl-CoA dehydrogenase
VLCAD
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REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT
LITERATURE
a very-long-chain acyl-CoA + electron-transfer flavoprotein = a very-long-chain trans-2,3-dehydroacyl-CoA + reduced electron-transfer flavoprotein
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PATHWAY SOURCE
PATHWAYS
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SYSTEMATIC NAME
IUBMB Comments
very-long-chain acyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase
Contains FAD as prosthetic group. One of several enzymes that catalyse the first step in fatty acids beta-oxidation. The enzyme is most active toward long-chain acyl-CoAs such as C14, C16 and C18, but is also active with very-long-chain acyl-CoAs up to 24 carbons. It shows no activity for substrates of less than 12 carbons. Its specific activity towards palmitoyl-CoA is more than 10-fold that of the long-chain acyl-CoA dehydrogenase [1]. cf. EC 1.3.8.1, short-chain acyl-CoA dehydrogenase, EC 1.3.8.7, medium-chain acyl-CoA dehydrogenase, and EC 1.3.8.8, long-chain acyl-CoA dehydrogenase.
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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
palmitoyl-CoA + electron-transfer flavoprotein
(2E)-2-hexadecenoyl-CoA + reduced electron-transfer flavoprotein
palmitoyl-CoA + electron-transfer flavoprotein
(2E)-2-hexadecenoyl-CoA + reduced electron-transfer flavoprotein
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-
-
-
?
palmitoyl-CoA + electron-transfer flavoprotein
(2E)-2-hexadecenoyl-CoA + reduced electron-transfer flavoprotein
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-
-
?
palmitoyl-CoA + electron-transfer flavoprotein
(2E)-2-hexadecenoyl-CoA + reduced electron-transfer flavoprotein
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-
-
-
?
palmitoyl-CoA + electron-transfer flavoprotein
(2E)-2-hexadecenoyl-CoA + reduced electron-transfer flavoprotein
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?
additional information
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acyl-CoA dehydrogenases, ACADs, constitute a family of FAD-dependent flavoproteins that catalyze the alpha,beta-dehydrogenation of thioester substrates
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additional information
?
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the enzyme is a cardiolipin-binding protein
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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
additional information
?
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acyl-CoA dehydrogenases, ACADs, constitute a family of FAD-dependent flavoproteins that catalyze the alpha,beta-dehydrogenation of thioester substrates
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COFACTOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
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ACTIVATING COMPOUND
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
additional information
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SIRT3 deacetylates and SIRT5 desuccinylates K299 which serves to stabilize the essential FAD cofactor in the active site of the enzyme
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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
Acidosis, Lactic
Fatty Acid oxidation disorder with secondary mitochondrial energy production defect: a case report.
acyl-coa dehydrogenase deficiency
Clinical features and mutations in seven Chinese patients with very long chain acyl-CoA dehydrogenase deficiency.
acyl-coa dehydrogenase deficiency
Gene symbol: ACADVL. Disease: Very long chain acyl-CoA dehydrogenase deficiency.
acyl-coa dehydrogenase deficiency
Newborn screening for disorders of fatty-acid oxidation: experience and recommendations from an expert meeting.
Biotinidase Deficiency
[The advisory report 'Neonatal screening' from the Health Council of The Netherlands]
Carcinogenesis
Differential gene expression identified in Uigur women cervical squamous cell carcinoma by suppression subtractive hybridization.
Cardiomyopathies
Clinical features and mutations in seven Chinese patients with very long chain acyl-CoA dehydrogenase deficiency.
Cardiomyopathies
Compared effects of missense mutations in Very-Long-Chain Acyl-CoA Dehydrogenase deficiency: Combined analysis by structural, functional and pharmacological approaches.
Cardiomyopathies
Molecular basis of human mitochondrial very-long-chain acyl-CoA dehydrogenase deficiency causing cardiomyopathy and sudden death in childhood.
Cardiomyopathies
Molecular heterogeneity in very-long-chain acyl-CoA dehydrogenase deficiency causing pediatric cardiomyopathy and sudden death.
Cardiomyopathies
Polymorphic ventricular tachycardia and abnormal Ca2+ handling in very-long-chain acyl-CoA dehydrogenase null mice.
Cardiomyopathies
Treatment of cardiomyopathy and rhabdomyolysis in long-chain fat oxidation disorders using an anaplerotic odd-chain triglyceride.
Cardiomyopathy, Hypertrophic
Very long chain acyl-CoA dehydrogenase deficiency: successful treatment of acute cardiomyopathy.
Death, Sudden, Cardiac
Polymorphic ventricular tachycardia and abnormal Ca2+ handling in very-long-chain acyl-CoA dehydrogenase null mice.
Fatty Liver
Combination of postmortem mass spectrometry imaging and genetic analysis reveals very long-chain acyl-CoA dehydrogenase deficiency in a case of infant death with liver steatosis.
Hepatic Encephalopathy
Compared effects of missense mutations in Very-Long-Chain Acyl-CoA Dehydrogenase deficiency: Combined analysis by structural, functional and pharmacological approaches.
Homocystinuria
[The advisory report 'Neonatal screening' from the Health Council of The Netherlands]
hydroxymethylglutaryl-coa lyase deficiency
[The advisory report 'Neonatal screening' from the Health Council of The Netherlands]
Hypoglycemia
Abnormal mitochondrial bioenergetics and heart rate dysfunction in mice lacking very-long-chain acyl-CoA dehydrogenase.
Hypoglycemia
Clinical features and mutations in seven Chinese patients with very long chain acyl-CoA dehydrogenase deficiency.
Hypoglycemia
Very long chain acyl-CoA dehydrogenase deficiency: successful treatment of acute cardiomyopathy.
long-chain acyl-coa dehydrogenase deficiency
Atypical presentation of VLCAD deficiency associated with a novel ACADVL splicing mutation.
long-chain acyl-coa dehydrogenase deficiency
Clinical features and mutations in seven Chinese patients with very long chain acyl-CoA dehydrogenase deficiency.
long-chain acyl-coa dehydrogenase deficiency
Clinical, Biochemical, and Molecular Features in 37 Saudi Patients with Very Long Chain Acyl CoA Dehydrogenase Deficiency.
long-chain acyl-coa dehydrogenase deficiency
Combination of postmortem mass spectrometry imaging and genetic analysis reveals very long-chain acyl-CoA dehydrogenase deficiency in a case of infant death with liver steatosis.
long-chain acyl-coa dehydrogenase deficiency
Familial very long chain acyl-CoA dehydrogenase deficiency as a cause of neonatal sudden infant death: improved survival by prompt diagnosis.
long-chain acyl-coa dehydrogenase deficiency
Gene symbol: ACADVL. Disease: Very long chain acyl-CoA dehydrogenase deficiency.
long-chain acyl-coa dehydrogenase deficiency
Identification of very-long-chain acyl-CoA dehydrogenase deficiency in three patients previously diagnosed with long-chain acyl-CoA dehydrogenase deficiency.
long-chain acyl-coa dehydrogenase deficiency
Management of fatty acid oxidation disorders: a survey of current treatment strategies.
long-chain acyl-coa dehydrogenase deficiency
Next generation sequencing as a follow-up test in an expanded newborn screening programme.
long-chain-3-hydroxyacyl-coa dehydrogenase deficiency
Disorders of muscle lipid metabolism: diagnostic and therapeutic challenges.
long-chain-3-hydroxyacyl-coa dehydrogenase deficiency
Heat stress deteriorates mitochondrial beta-oxidation of long-chain fatty acids in cultured fibroblasts with fatty acid beta-oxidation disorders.
long-chain-3-hydroxyacyl-coa dehydrogenase deficiency
Management of fatty acid oxidation disorders: a survey of current treatment strategies.
Maple Syrup Urine Disease
[The advisory report 'Neonatal screening' from the Health Council of The Netherlands]
medium-chain acyl-coa dehydrogenase deficiency
Management of fatty acid oxidation disorders: a survey of current treatment strategies.
medium-chain acyl-coa dehydrogenase deficiency
Newborn screening for disorders of fatty-acid oxidation: experience and recommendations from an expert meeting.
medium-chain acyl-coa dehydrogenase deficiency
Simultaneous analysis of plasma free fatty acids and their 3-hydroxy analogs in fatty acid beta-oxidation disorders.
medium-chain acyl-coa dehydrogenase deficiency
[The advisory report 'Neonatal screening' from the Health Council of The Netherlands]
Metabolic Diseases
Clinical features and mutations in seven Chinese patients with very long chain acyl-CoA dehydrogenase deficiency.
methylcrotonoyl-coa carboxylase deficiency
[The advisory report 'Neonatal screening' from the Health Council of The Netherlands]
Muscle Weakness
Treatment of cardiomyopathy and rhabdomyolysis in long-chain fat oxidation disorders using an anaplerotic odd-chain triglyceride.
Muscular Diseases
Clinical features and mutations in seven Chinese patients with very long chain acyl-CoA dehydrogenase deficiency.
Muscular Diseases
Compared effects of missense mutations in Very-Long-Chain Acyl-CoA Dehydrogenase deficiency: Combined analysis by structural, functional and pharmacological approaches.
Muscular Diseases
Myopathy in very-long-chain acyl-CoA dehydrogenase deficiency: clinical and biochemical differences with the fatal cardiac phenotype.
Myoglobinuria
A novel mutation of late-onset very-long-chain acyl-CoA dehydrogenase deficiency.
Neoplasms
Loss of heterozygosity of 17p13, with possible involvement of ACADVL and ALOX15B, in the pathogenesis of adrenocortical tumors.
Pericardial Effusion
Very long chain acyl-CoA dehydrogenase deficiency: successful treatment of acute cardiomyopathy.
phosphatidate phosphatase deficiency
Disorders of muscle lipid metabolism: diagnostic and therapeutic challenges.
Protein Deficiency
Disorders of muscle lipid metabolism: diagnostic and therapeutic challenges.
Protein Deficiency
Electrospray tandem mass spectrometry for analysis of acylcarnitines in dried postmortem blood specimens collected at autopsy from infants with unexplained cause of death.
Protein Deficiency
Heat stress deteriorates mitochondrial beta-oxidation of long-chain fatty acids in cultured fibroblasts with fatty acid beta-oxidation disorders.
Protein Deficiency
Management of fatty acid oxidation disorders: a survey of current treatment strategies.
Rhabdomyolysis
A novel mutation of late-onset very-long-chain acyl-CoA dehydrogenase deficiency.
Rhabdomyolysis
Disorders of muscle lipid metabolism: diagnostic and therapeutic challenges.
Rhabdomyolysis
Intermittent rhabdomyolysis with adult onset associated with a mutation in the ACADVL gene.
Rhabdomyolysis
Treatment of cardiomyopathy and rhabdomyolysis in long-chain fat oxidation disorders using an anaplerotic odd-chain triglyceride.
Rhabdomyolysis
[A case of very-long-chain acyl-CoA dehydrogenase deficiency with adolescent onset being diagnosed by immunostain of biopsy muscle]
Rhabdomyolysis
[Very-long-chain acyl-CoA dehydrogenase deficiency presenting with recurrent rhabdomyolysis in an adult]
Tachycardia, Ventricular
Polymorphic ventricular tachycardia and abnormal Ca2+ handling in very-long-chain acyl-CoA dehydrogenase null mice.
very-long-chain acyl-coa dehydrogenase deficiency
A novel mutation of late-onset very-long-chain acyl-CoA dehydrogenase deficiency.
very-long-chain acyl-coa dehydrogenase deficiency
Clear correlation of genotype with disease phenotype in very-long-chain acyl-CoA dehydrogenase deficiency.
very-long-chain acyl-coa dehydrogenase deficiency
Compared effects of missense mutations in Very-Long-Chain Acyl-CoA Dehydrogenase deficiency: Combined analysis by structural, functional and pharmacological approaches.
very-long-chain acyl-coa dehydrogenase deficiency
Development of a new enzymatic diagnosis method for very-long-chain Acyl-CoA dehydrogenase deficiency by detecting 2-hexadecenoyl-CoA production and its application in tandem mass spectrometry-based selective screening and newborn screening in Japan.
very-long-chain acyl-coa dehydrogenase deficiency
DNA-based prenatal diagnosis for very-long-chain acyl-CoA dehydrogenase deficiency.
very-long-chain acyl-coa dehydrogenase deficiency
Electrospray tandem mass spectrometry for analysis of acylcarnitines in dried postmortem blood specimens collected at autopsy from infants with unexplained cause of death.
very-long-chain acyl-coa dehydrogenase deficiency
Expanded newborn screening in New South Wales: missed cases.
very-long-chain acyl-coa dehydrogenase deficiency
Fatty Acid oxidation disorder with secondary mitochondrial energy production defect: a case report.
very-long-chain acyl-coa dehydrogenase deficiency
Fatty acid oxidation disorders: outcome and long-term prognosis.
very-long-chain acyl-coa dehydrogenase deficiency
Genotype-phenotype correlations: sudden death in an infant with very-long-chain acyl-CoA dehydrogenase deficiency.
very-long-chain acyl-coa dehydrogenase deficiency
Heat stress deteriorates mitochondrial beta-oxidation of long-chain fatty acids in cultured fibroblasts with fatty acid beta-oxidation disorders.
very-long-chain acyl-coa dehydrogenase deficiency
High-resolution melting analysis, a simple and effective method for reliable mutation scanning and frequency studies in the ACADVL gene.
very-long-chain acyl-coa dehydrogenase deficiency
Identification and characterization of temperature-sensitive mild mutations in three Japanese patients with nonsevere forms of very-long-chain acyl-CoA dehydrogenase deficiency.
very-long-chain acyl-coa dehydrogenase deficiency
Identification of very-long-chain acyl-CoA dehydrogenase deficiency in three patients previously diagnosed with long-chain acyl-CoA dehydrogenase deficiency.
very-long-chain acyl-coa dehydrogenase deficiency
Impact of NBS for VLCAD deficiency on genetic, enzymatic and clinical outcomes.
very-long-chain acyl-coa dehydrogenase deficiency
Management and diagnosis of mitochondrial fatty acid oxidation disorders: focus on very-long-chain acyl-CoA dehydrogenase deficiency.
very-long-chain acyl-coa dehydrogenase deficiency
Management of fatty acid oxidation disorders: a survey of current treatment strategies.
very-long-chain acyl-coa dehydrogenase deficiency
Medium-chain triglycerides impair lipid metabolism and induce hepatic steatosis in very long-chain acyl-CoA dehydrogenase (VLCAD)-deficient mice.
very-long-chain acyl-coa dehydrogenase deficiency
Molecular and cellular pathology of very-long-chain acyl-CoA dehydrogenase deficiency.
very-long-chain acyl-coa dehydrogenase deficiency
Molecular basis of human mitochondrial very-long-chain acyl-CoA dehydrogenase deficiency causing cardiomyopathy and sudden death in childhood.
very-long-chain acyl-coa dehydrogenase deficiency
Molecular heterogeneity in very-long-chain acyl-CoA dehydrogenase deficiency causing pediatric cardiomyopathy and sudden death.
very-long-chain acyl-coa dehydrogenase deficiency
MS/MS-based newborn and family screening detects asymptomatic patients with very-long-chain acyl-CoA dehydrogenase deficiency.
very-long-chain acyl-coa dehydrogenase deficiency
Myopathy in very-long-chain acyl-CoA dehydrogenase deficiency: clinical and biochemical differences with the fatal cardiac phenotype.
very-long-chain acyl-coa dehydrogenase deficiency
Newborn screening for disorders of fatty-acid oxidation: experience and recommendations from an expert meeting.
very-long-chain acyl-coa dehydrogenase deficiency
Novel missense mutations in the first Chinese patient with very-long-chain acyl-CoA dehydrogenase deficiency.
very-long-chain acyl-coa dehydrogenase deficiency
Novel mutation of early, perinatal-onset, myopathic-type very-long-chain acyl-CoA dehydrogenase deficiency.
very-long-chain acyl-coa dehydrogenase deficiency
Oxidation of unsaturated fatty acids by human fibroblasts with very-long-chain acyl-CoA dehydrogenase deficiency: aspects of substrate specificity and correlation with clinical phenotype.
very-long-chain acyl-coa dehydrogenase deficiency
Pitfalls of neonatal screening for very-long-chain acyl-CoA dehydrogenase deficiency using tandem mass spectrometry.
very-long-chain acyl-coa dehydrogenase deficiency
Rare Korean Cases of Very-long-chain Acyl-CoA Dehydrogenase Deficiency with a Novel Recurrent Mutation.
very-long-chain acyl-coa dehydrogenase deficiency
Simultaneous analysis of plasma free fatty acids and their 3-hydroxy analogs in fatty acid beta-oxidation disorders.
very-long-chain acyl-coa dehydrogenase deficiency
Treatment of cardiomyopathy and rhabdomyolysis in long-chain fat oxidation disorders using an anaplerotic odd-chain triglyceride.
very-long-chain acyl-coa dehydrogenase deficiency
Very-long-chain acyl-CoA dehydrogenase deficiency: molecular genetics of a mitochondrial membrane enzyme.
very-long-chain acyl-coa dehydrogenase deficiency
[A case of very-long-chain acyl-CoA dehydrogenase deficiency with adolescent onset being diagnosed by immunostain of biopsy muscle]
very-long-chain acyl-coa dehydrogenase deficiency
[A two-year-old infant with a myopathic form of very-long-chain Acyl-CoA dehydrogenase deficiency]
very-long-chain acyl-coa dehydrogenase deficiency
[Novel nutritional management regimen for very long-chain acyl-CoA dehydrogenase deficiency]
very-long-chain acyl-coa dehydrogenase deficiency
[The advisory report 'Neonatal screening' from the Health Council of The Netherlands]
very-long-chain acyl-coa dehydrogenase deficiency
[Very-long-chain acyl-CoA dehydrogenase deficiency presenting with recurrent rhabdomyolysis in an adult]
very-long-chain acyl-coa dehydrogenase deficiency
[Very-long-chain acyl-CoA dehydrogenase deficiency]
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ORGANISM
COMMENTARY
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
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SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
SOURCE
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LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY
GeneOntology No.
LITERATURE
SOURCE
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GENERAL INFORMATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
malfunction
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phosphorylation of VLCAD at Ser586 is inhibited in myofibroblasts, resulting in a significant loss of enzyme activity coupled with lipid peroxidation.Thus Ser586 represents a critical site for VLCAD activity, whose dysregulation might contribute to the progression of idiopathic pulmonary fibrosis, IPF, a chronic interstitial lung disease, and other oxidative-stress mediated diseases
physiological function
physiological function
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VLCAD is a rate-limiting enzyme in fatty acid beta-oxidation and is regulated by phosphorylation at Ser586
physiological function
the enzyme is a diet-sensitive source of mitochondrial reactive oxygen species
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UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
Sequence
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PDB
SCOP
CATH
UNIPROT
ORGANISM
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MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
180000
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about, size-exclusion chromatography coupled with in-line static light-scattering, refractive-index and ultraviolet measurements
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SUBUNIT
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
tetramer
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4 x 45000, about, size-exclusion chromatography coupled with in-line static light-scattering, refractive-index and ultraviolet measurements
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POSTTRANSLATIONAL MODIFICATION
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
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CRYSTALLIZATION/commentary
ORGANISM
UNIPROT
LITERATURE
purified recombinant cVLCAD, hanging-drop vapour-diffusion method, 0.002 ml of 12 mg/ml protein in 20 mM Tris-HCl pH 8.0, 150 mM NaCl are mixed with 0.002 ml of reservoir solution containing 100 mM Tris-HCl, pH 8.0, 150 mM NaCl, 200 mM magnesium formate and 13% PEG 3350, 16°C, 3 days, X-ray diffraction structure determination and analysis at 1.8-2.6 A resolution, molecular replacement
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PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
S586A
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naturally occuring mutation leading to phosphorylation of VLCAD at Ser586 is inhibited in myofibroblasts, resulting in a significant loss of enzyme activity coupled with lipid peroxidation. The S586A mutant shows a significant reduction in electron transfer activity
T409M
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the mutation is associated with partial carnitine palmitoyltransferase II deficiency
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PURIFICATION/commentary
ORGANISM
UNIPROT
LITERATURE
recombinant His8-tagged VLCAD from Escherichia coli strain BL21 (DE3) by nickel affinity chromatography, anion exchange chromatography, and gel filtration
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CLONED/commentary
ORGANISM
UNIPROT
LITERATURE
cloning from cDNA library, His8-tagged VLCAD expression in Escherichia coli strain BL21 (DE3)
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REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Primassin, S.; Ter Veld, F.; Mayatepek, E.; Spiekerkoetter, U.
Carnitine supplementation induces acylcarnitine production in tissues of very long-chain acyl-CoA dehydrogenase-deficient mice, without replenishing low free carnitine
Pediatr. Res.
63
632-637
2008
Mus musculus
brenda
Tajima, G.; Sakura, N.; Shirao, K.; Okada, S.; Tsumura, M.; Nishimura, Y.; Ono, H.; Hasegawa, Y.; Hata, I.; Naito, E.; Yamaguchi, S.; Shigematsu, Y.; Kobayashi, M.
Development of a new enzymatic diagnosis method for very-long-chain acyl-CoA dehydrogenase deficiency by detecting 2-hexadecenoyl-CoA production and its application in tandem mass spectrometry-based selective screening and newborn screening in Japan
Pediatr. Res.
64
667-672
2008
Homo sapiens
brenda
Li, Z.; Zhai, Y.; Fang, J.; Zhou, Q.; Geng, Y.; Sun, F.
Purification, crystallization and preliminary crystallographic analysis of very-long-chain acyl-CoA dehydrogenase from Caenorhabditis elegans
Acta Crystallogr. Sect. F
66
426-430
2010
Caenorhabditis elegans
brenda
Kabuyama, Y.; Suzuki, T.; Nakazawa, N.; Yamaki, J.; Homma, M.; Homma, Y.
Dysregulation of very long chain acyl-CoA dehydrogenase coupled with lipid peroxidation
Am. J. Physiol. Cell Physiol.
298
C107-C113
2010
Homo sapiens
brenda
Zhang, Y.; Bharathi, S.S.; Rardin, M.J.; Uppala, R.; Verdin, E.; Gibson, B.W.; Goetzman, E.S.
SIRT3 and SIRT5 regulate the enzyme activity and cardiolipin binding of very long-chain acyl-CoA dehydrogenase
PLoS ONE
10
e0122297
2015
Homo sapiens (P49748)
brenda
Isackson, P.; Sutton, K.; Hostetler, K.; Vladutiu, G.
Novel mutations in the gene encoding very long-chain acyl-CoA dehydrogenase identified in patients with partial carnitine palmitoyltransferase II deficiency
Muscle Nerve
47
224-229
2013
Homo sapiens (P49748)
brenda
Cardoso, A.; Kakimoto, P.; Kowaltowski, A.
Diet-sensitive sources of reactive oxygen species in liver mitochondria: role of very long chain acyl-CoA dehydrogenases
PLoS ONE
8
e77088
2013
Mus musculus (P50544)
brenda
Kakimoto, P.A.; Tamaki, F.K.; Cardoso, A.R.; Marana, S.R.; Kowaltowski, A.J.
H2O2 release from the very long chain acyl-CoA dehydrogenase
Redox Biol.
4
375-380
2015
Homo sapiens (P49748)
brenda
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