Human acyl-Coenzyme A dehydrogenase, very long chain ELISA Kit | ACADVL elisa kit
Human 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; Lipid Metabolism - fatty acid; EC 1.3.8.9; Oxidoreductase
Chromosomal Location of Human Ortholog: 17p13.1
Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial inner membrane; cytoplasm; nucleolus; nucleus
Molecular Function: acyl-CoA dehydrogenase activity; FAD binding; long-chain-acyl-CoA dehydrogenase activity
Biological Process: cellular protein metabolic process; fatty acid beta-oxidation; unfolded protein response, activation of signaling protein activity; epithelial cell differentiation; unfolded protein response; cellular lipid metabolic process; thermoregulation; fatty acid beta-oxidation using acyl-CoA dehydrogenase; negative regulation of fatty acid oxidation; negative regulation of fatty acid biosynthetic process; energy derivation by oxidation of organic compounds
Disease: Acyl-coa Dehydrogenase, Very Long-chain, Deficiency Of
Research Articles on ACADVL
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Product Notes
The Human ACADVL acadvl (Catalog #AAA9316130) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9316130 ELISA Kit recognizes Human ACADVL. It is sometimes possible for the material contained within the vial of "acyl-Coenzyme A dehydrogenase, very 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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