Human acyl-Coenzyme A dehydrogenase, long chain ELISA Kit | ACADL elisa kit
Human Long-chain specific acyl-CoA dehydrogenase, mitochondrial, ACADL ELISA Kit
NCBI and Uniprot Product Information
NCBI Description
The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family, which is a family of mitochondrial flavoenzymes involved in fatty acid and branched chain amino-acid metabolism. This protein is one of the four enzymes that catalyze the initial step of mitochondrial beta-oxidation of straight-chain fatty acid. Defects in this gene are the cause of long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycemia. [provided by RefSeq, Jul 2008]
Uniprot Description
ACADL: Defects in ACADL are a cause of acyl-CoA dehydrogenase very long-chain deficiency (ACADVLD). An inborn error of mitochondrial fatty acid beta-oxidation which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form characterized by early onset, high mortality and high incidence of cardiomyopathy; a milder childhood form with later onset, characterized by hypoketotic hypoglycemia, low mortality and rare cardiomyopathy; an adult form, with isolated skeletal muscle involvement, rhabdomyolysis and myoglobinuria, usually triggered by exercise or fasting. Belongs to the acyl-CoA dehydrogenase family.
Protein type: Lipid Metabolism - fatty acid; EC 1.3.8.8; Oxidoreductase; Mitochondrial
Chromosomal Location of Human Ortholog: 2q34
Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial membrane; cytoplasm
Molecular Function: palmitoyl-CoA oxidase activity; acyl-CoA dehydrogenase activity; FAD binding; acyl-CoA binding; long-chain-acyl-CoA dehydrogenase activity
Biological Process: carnitine metabolic process, CoA-linked; fatty acid beta-oxidation; cellular lipid catabolic process; carnitine catabolic process; thermoregulation; cellular lipid metabolic process; fatty acid beta-oxidation using acyl-CoA dehydrogenase; negative regulation of fatty acid oxidation; negative regulation of fatty acid biosynthetic process; long-chain fatty acid catabolic process; protein homotetramerization
Research Articles on ACADL
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Product Notes
The Human ACADL acadl (Catalog #AAA9329088) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9329088 ELISA Kit recognizes Human ACADL. It is sometimes possible for the material contained within the vial of "acyl-Coenzyme A dehydrogenase, long chain, ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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