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

SKU: AE34053HU

Size: 96T. Other sizes are also available.

Species Reactivity: Human (Homo sapiens)

UniProt: Q9HCC0

Abbreviation: MCCC2

Alternative Names: MCCB; biotin carboxylase|non-biotin containing subunit of 3-methylcrotonyl-CoA carboxylase

Application: ELISA

Range: 0.312-20 ng/mL

Sensitivity: 0.141 ng/mL

Test principle: This assay 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.

Product Overview: 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.

Stability: The stability of ELISA kit is determined by the loss rate of activity. The loss rate of this kit is less than 5% within the expiration date under appropriate storage condition. The loss rate was determined by accelerated thermal degradation test. Keep the kit at 37°C for 4 and 7 days, and compare O.D.values of the kit kept at 37°C with that of at recommended temperature. (referring from China Biological Products Standard, which was calculated by the Arrhenius equation. For ELISA kit, 4 days storage at 37°C can be considered as 6 months at 2 - 8°C, which means 7 days at 37°C equaling 12 months at 2 - 8°C).