Molecular weight (DA)
72kD
Immunogen
Synthesized peptide derived from human protein . at AA range: 471-520
Specificity
ACADV Polyclonal Antibody detects endogenous levels of protein.
Formulation
Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.
Dilution rate
WB 1:500-2000 ELISA 1:5000-20000
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Background
acyl-CoA dehydrogenase, very long chain(ACADVL) Homo sapiens The protein encoded by this gene is targeted to the inner mitochondrial membrane where it catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in this gene product reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jul 2008],
Function
catalytic activity:Acyl-CoA + ETF = 2,3-dehydroacyl-CoA + reduced ETF.,cofactor:FAD.,disease:Defects in ACADVL are the cause of very long chain acyl-CoA dehydrogenase deficiency (VLCAD deficiency) [MIM:201475]. VLCAD deficiency is an autosomal recessive disease which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form, with early onset, high mortality, and high incidence of cardiomyopathy; a milder childhood form, with later onset, usually with hypoketotic hypoglycemia as the main presenting feature, low mortality, and rare cardiomyopathy; and an adult form, with isolated skeletal muscle involvement, rhabdomyolysis, and myoglobinuria, usually triggered by exercise or fasting.,function:Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-
Protein name
Very long-chain specific acyl-CoA dehydrogenase, mitochondrial (VLCAD) (EC 1.3.8.9)
Fields
>>Fatty acid degradation;>>Metabolic pathways;>>Fatty acid metabolism;>>Alcoholic liver disease
Human protein sequence Database
P49748
Mouse protein sequence database
P50544
Rat protein sequence database
P45953
Cellular localization
Mitochondrion inner membrane ; Peripheral membrane protein .; [Isoform 2]: Mitochondrion inner membrane ; Peripheral membrane protein .
Tissue expression
Adipose tissue,Liver,Lung,Pancreas,Peripheral blood,Placenta,
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