Human Long chain specific acyl CoA dehydrogenase, mitochondrial (ACADL) ELISA Kit | ACADL elisa kit

Human Long chain specific acyl CoA dehydrogenase, mitochondrial (ACADL) ELISA Kit

Cat. Num. AAA7201517

• Gene Names: ACADL; LCAD; ACAD4
• Reactivity: Human
• Synonyms: Long chain specific acyl CoA dehydrogenase; mitochondrial (ACADL); Human Long chain specific acyl CoA dehydrogenase; mitochondrial (ACADL) ELISA Kit; ACADL elisa kit

• Reactivity: Human

• Samples: Serum, plasma, Cell Culture Supernatants, body fluid and tissue homogenate
• Assay Type: Competitive
• Detection Range: 5.0-100ng/mL
• Sensitivity: 0.1ng/mL
• Preparation and Storage: Store all reagents at 2-8 degree C.

Typical Testing Data/Standard Curve (for reference only)

Related Product Information for ACADL elisa kit

Intended Uses||This ACADL ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Human ACADL. This ELISA kit for research use only!

Principle of the Assay: ACADL ELISA kit applies the competitive enzyme immunoassay technique utilizing a monoclonal anti-ACADL antibody and an ACADL-HRP conjugate. The assay sample and buffer are incubated together with ACADL-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the ACADL concentration since ACADL from samples and ACADL-HRP conjugate compete for the anti-ACADL antibody binding site. Since the number of sites is limited, as more sites are occupied by ACADL from the sample, fewer sites are left to bind ACADL-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The ACADL concentration in each sample is interpolated from this standard curve.

NCBI and Uniprot Product Information

• NCBI GI #: 4501857
• NCBI GeneID: 33
• NCBI Accession #: NP_001599.1
• NCBI GenBank Nucleotide #: NM_001608.3
• Molecular Weight: 47,656 Da
• NCBI Official Full Name: long-chain specific acyl-CoA dehydrogenase, mitochondrial
• NCBI Official Synonym Full Names: acyl-CoA dehydrogenase, long chain
• NCBI Official Symbol: ACADL
• NCBI Official Synonym Symbols: LCAD; ACAD4
• NCBI Protein Information: long-chain specific acyl-CoA dehydrogenase, mitochondrial; acyl-Coenzyme A dehydrogenase, long chain
• UniProt Protein Name: Long-chain specific acyl-CoA dehydrogenase, mitochondrial
• Protein Family: Long-chain specific acyl-CoA dehydrogenase
• UniProt Gene Name: ACADL
• UniProt Synonym Gene Names: LCAD
• UniProt Entry Name: ACADL_HUMAN

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; Mitochondrial; Oxidoreductase

Chromosomal Location of Human Ortholog: 2q34

Cellular Component: mitochondrion; mitochondrial matrix; cytoplasm; mitochondrial membrane

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 biosynthetic process; negative regulation of fatty acid oxidation; long-chain fatty acid catabolic process; protein homotetramerization