Porcine Long-Chain Specific Acyl-CoA Dehydrogenase, Mitochondrial (ACADL) ELISA Kit | ACADL elisa kit
Porcine 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; Oxidoreductase; Mitochondrial; EC 1.3.8.8
Chromosomal Location of Human Ortholog: 2q34
Cellular Component: mitochondrial matrix; mitochondrion
Molecular Function: acyl-CoA binding; acyl-CoA dehydrogenase activity; electron carrier activity; FAD binding; long-chain-acyl-CoA dehydrogenase activity; palmitoyl-CoA oxidase activity
Biological Process: carnitine catabolic process; carnitine metabolic process, CoA-linked; cellular lipid catabolic process; fatty acid beta-oxidation; fatty acid beta-oxidation using acyl-CoA dehydrogenase; lipid homeostasis; long-chain fatty acid catabolic process; negative regulation of fatty acid biosynthetic process; negative regulation of fatty acid oxidation; thermoregulation
Research Articles on ACADL
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Product Notes
The Porcine ACADL acadl (Catalog #AAA9370607) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9370607 ELISA Kit recognizes Porcine ACADL. It is sometimes possible for the material contained within the vial of "Long-Chain Specific Acyl-CoA Dehydrogenase, Mitochondrial (ACADL), 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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