Human Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial (MCCC2) ELISA Kit

MCCC2 is the small subunit of 3-methylcrotonyl-CoA carboxylase. This enzyme functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. Mutations in this gene are associated with 3-Methylcrotonylglycinuria, an autosomal recessive disorder of leucine catabolism. Defects in MCCC2 are the cause of methylcrotonoyl-CoA carboxylase deficiency type 2 (MCC2 deficiency) . MCC2 deficiency is an autosomal recessive disorder of leucine catabolism. The phenotype is variable, ranging from neonatal onset with severe neurological involvement to asymptomatic adults. There is a characteristic organic aciduria with massive excretion of 3- hydroxyisovaleric acid and 3-methylcrotonylglycine, usually in combination with a severe secondary carnitine deficiency.

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Human Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial (MCCC2) ELISA Kit has high sensitivity and excellent specificity for detection of Human MCCC2. No significant cross-reactivity or interference between Human MCCC2 and analogues was observed.

This Human Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial (MCCC2) ELISA Kit employs a two-site sandwich ELISA to quantitate MCCC2 in samples. An antibody specific for MCCC2 has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and anyMCCC2 present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for MCCC2 is added to the wells. After washing, Streptavidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of MCCC2 bound in the initial step. The color development is stopped and the intensity of the color is measured.

• Do not mix components from different kit lots or use reagents beyond the kit expiration date.• Allow all reagents to warm to room temperature for at least 30 minutes before opening.• Pre-rinse the pipet tip with reagent, use fresh pipet tips for each sample, standard and reagent to avoid contamination.• Unused wells must be kept desiccated at 4 °C in the sealed bag provided.• Mix Thoroughly is very important for the result. It is recommended using low frequency oscillator or slight hand shaking every 10 minutes.• It is recommended that all samples and standards be assayed in duplicate or triplicate.