Cavy Very Long-Chain Specific Acyl-CoA Dehydrogenase, Mitochondrial (ACADVL) ELISA Kit | ACADVL elisa kit
Cavy Very Long-Chain Specific Acyl-CoA Dehydrogenase, Mitochondrial (ACADVL) ELISA Kit
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
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 acyl-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: Mitochondrial; EC 1.3.8.9; Oxidoreductase; Lipid Metabolism - fatty acid
Chromosomal Location of Human Ortholog: 17p13.1
Cellular Component: cytoplasm; mitochondrial inner membrane; mitochondrial matrix; 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: cellular lipid metabolic process; cellular protein metabolic process; energy derivation by oxidation of organic compounds; epithelial cell differentiation; fatty acid beta-oxidation; fatty acid beta-oxidation using acyl-CoA dehydrogenase; lipid homeostasis; negative regulation of fatty acid biosynthetic process; negative regulation of fatty acid oxidation; thermoregulation; unfolded protein response; very-long-chain fatty acid catabolic process
Disease: Acyl-coa Dehydrogenase, Very Long-chain, Deficiency Of
Research Articles on ACADVL
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Product Notes
The Cavy ACADVL acadvl (Catalog #AAA9357050) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9357050 ELISA Kit recognizes Cavy ACADVL. It is sometimes possible for the material contained within the vial of "Very Long-Chain Specific Acyl-CoA Dehydrogenase, Mitochondrial (ACADVL), 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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