BRENDA - Enzyme Database show
show all sequences of 1.3.8.9

Dysregulation of very long chain acyl-CoA dehydrogenase coupled with lipid peroxidation

Kabuyama, Y.; Suzuki, T.; Nakazawa, N.; Yamaki, J.; Homma, M.; Homma, Y.; Am. J. Physiol. Cell Physiol. 298, C107-C113 (2010)

Data extracted from this reference:

Cloned(Commentary)
Commentary
Organism
expression of wild-type enzyme and mutant S586A in HEK293 cells
Homo sapiens
Engineering
Amino acid exchange
Commentary
Organism
S586A
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
Homo sapiens
Localization
Localization
Commentary
Organism
GeneOntology No.
Textmining
mitochondrion
-
Homo sapiens
5739
-
Organism
Organism
Primary Accession No. (UniProt)
Commentary
Textmining
Homo sapiens
-
-
-
Posttranslational Modification
Posttranslational Modification
Commentary
Organism
phosphoprotein
phosphorylation of Ser586 is essential for VLCAD function
Homo sapiens
Source Tissue
Source Tissue
Commentary
Organism
Textmining
LF2 cell
-
Homo sapiens
-
myofibroblast
-
Homo sapiens
-
TIG-7 cell
-
Homo sapiens
-
Cloned(Commentary) (protein specific)
Commentary
Organism
expression of wild-type enzyme and mutant S586A in HEK293 cells
Homo sapiens
Engineering (protein specific)
Amino acid exchange
Commentary
Organism
S586A
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
Homo sapiens
Localization (protein specific)
Localization
Commentary
Organism
GeneOntology No.
Textmining
mitochondrion
-
Homo sapiens
5739
-
Posttranslational Modification (protein specific)
Posttranslational Modification
Commentary
Organism
phosphoprotein
phosphorylation of Ser586 is essential for VLCAD function
Homo sapiens
Source Tissue (protein specific)
Source Tissue
Commentary
Organism
Textmining
LF2 cell
-
Homo sapiens
-
myofibroblast
-
Homo sapiens
-
TIG-7 cell
-
Homo sapiens
-
General Information
General Information
Commentary
Organism
malfunction
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
Homo sapiens
physiological function
VLCAD is a rate-limiting enzyme in fatty acid beta-oxidation and is regulated by phosphorylation at Ser586
Homo sapiens
General Information (protein specific)
General Information
Commentary
Organism
malfunction
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
Homo sapiens
physiological function
VLCAD is a rate-limiting enzyme in fatty acid beta-oxidation and is regulated by phosphorylation at Ser586
Homo sapiens
Other publictions for EC 1.3.8.9
No.
1st author
Pub Med
title
organims
journal
volume
pages
year
Activating Compound
Application
Cloned(Commentary)
Crystallization (Commentary)
Engineering
General Stability
Inhibitors
KM Value [mM]
Localization
Metals/Ions
Molecular Weight [Da]
Natural Substrates/ Products (Substrates)
Organic Solvent Stability
Organism
Oxidation Stability
Posttranslational Modification
Purification (Commentary)
Reaction
Renatured (Commentary)
Source Tissue
Specific Activity [micromol/min/mg]
Storage Stability
Substrates and Products (Substrate)
Subunits
Temperature Optimum [°C]
Temperature Range [°C]
Temperature Stability [°C]
Turnover Number [1/s]
pH Optimum
pH Range
pH Stability
Cofactor
Ki Value [mM]
pI Value
IC50 Value
Activating Compound (protein specific)
Application (protein specific)
Cloned(Commentary) (protein specific)
Cofactor (protein specific)
Crystallization (Commentary) (protein specific)
Engineering (protein specific)
General Stability (protein specific)
IC50 Value (protein specific)
Inhibitors (protein specific)
Ki Value [mM] (protein specific)
KM Value [mM] (protein specific)
Localization (protein specific)
Metals/Ions (protein specific)
Molecular Weight [Da] (protein specific)
Natural Substrates/ Products (Substrates) (protein specific)
Organic Solvent Stability (protein specific)
Oxidation Stability (protein specific)
Posttranslational Modification (protein specific)
Purification (Commentary) (protein specific)
Renatured (Commentary) (protein specific)
Source Tissue (protein specific)
Specific Activity [micromol/min/mg] (protein specific)
Storage Stability (protein specific)
Substrates and Products (Substrate) (protein specific)
Subunits (protein specific)
Temperature Optimum [°C] (protein specific)
Temperature Range [°C] (protein specific)
Temperature Stability [°C] (protein specific)
Turnover Number [1/s] (protein specific)
pH Optimum (protein specific)
pH Range (protein specific)
pH Stability (protein specific)
pI Value (protein specific)
Expression
General Information
General Information (protein specific)
Expression (protein specific)
KCat/KM [mM/s]
KCat/KM [mM/s] (protein specific)
739412
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Isackson
Novel mutations in the gene en ...
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Muscle Nerve
47
224-229
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741280
Cardoso
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Mus musculus
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2013
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1
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1
1
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710755
Li
Purification, crystallization ...
Caenorhabditis elegans
Acta Crystallogr. Sect. F
66
426-430
2010
-
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1
1
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1
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3
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1
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723908
Kabuyama
Dysregulation of very long cha ...
Homo sapiens
Am. J. Physiol. Cell Physiol.
298
C107-C113
2010
-
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1
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1
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1
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-
1
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1
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3
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2
2
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689339
Primassin
Carnitine supplementation indu ...
Mus musculus
Pediatr. Res.
63
632-637
2008
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1
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1
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689340
Tajima
Development of a new enzymatic ...
Homo sapiens
Pediatr. Res.
64
667-672
2008
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3
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1
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