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Very long-chain specific acyl-CoA dehydrogenase

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Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accommodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons (By similarity).

Below are the list of possible Very long-chain specific acyl-CoA dehydrogenase products. If you cannot find the target and/or product is not available in our catalog, please click here to contact us and request the product or submit your request for custom elisa kit production, custom recombinant protein production or custom antibody production. Custom ELISA Kits, Recombinant Proteins and Antibodies can be designed, manufactured and produced according to the researcher's specifications.
 

Very long-chain specific acyl-CoA dehydrogenase

 Very long-chain specific acyl-CoA dehydrogenase ELISA Kit
 Very long-chain specific acyl-CoA dehydrogenase Recombinant
 Very long-chain specific acyl-CoA dehydrogenase Antibody
Also known as Very long-chain specific acyl-CoA dehydrogenase, mitochondrial (MVLCAD) (VLCAD).
ACADVL: Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accommodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons. Defects in ACADVL are the cause of acy
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l-CoA dehydrogenase very long chain deficiency (ACADVLD). ACADVLD 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. Belongs to the acyl-CoA dehydrogenase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 1.3.8.9; Lipid Metabolism - fatty acid; Mitochondrial; Oxidoreductase

Cellular Component: cytoplasm; mitochondrial inner membrane; mitochondrion; nucleolus; nucleus

Molecular Function: acyl-CoA binding; acyl-CoA dehydrogenase activity; electron carrier activity; FAD binding; long-chain-acyl-CoA dehydrogenase activity; very-long-chain-acyl-CoA dehydrogenase activity

Biological Process: fatty acid beta-oxidation; fatty acid beta-oxidation using acyl-CoA dehydrogenase; fatty acid catabolic process; lipid homeostasis; negative regulation of fatty acid biosynthetic process; negative regulation of fatty acid oxidation; response to cold; thermoregulation; very-long-chain fatty acid catabolic process
 Acadvl ELISA Kit
 Acadvl Recombinant
 Acadvl Antibody
 Vlcad ELISA Kit
 Vlcad Recombinant
 Vlcad Antibody
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