Human Methylcrotonoyl-CoA carboxylase subunit alpha; mitochondrial (MCCC1) ELISA kit; DKFZp686B20267; FLJ25545; MCC-B; MCCA; 3-methylcrotonyl-CoA carboxylase biotin-containing subunit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha; methylcrotonoyl-Coenzyme A carboxylase 1 (alpha)

For Research Use Only. Not for use in diagnostic procedures.

This assay has high sensitivity and excellent specificity for detection of human MCCC1. No significant cross-reactivity or interference between human MCCC1 and analogues was observed.

Unopened test kits should be stored at 2 to 8 degree C upon receipt. Please refer to pdf manual for further storage instructions.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual.

Small volumes of MCCC1 elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS916524 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the methylcrotonoyl-Coenzyme A carboxylase 1 (alpha) (MCCC1) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing MCCC1. The ELISA analytical biochemical technique of the MBS916524 kit is based on MCCC1 antibody-MCCC1 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect MCCC1 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, MCCC1. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

Principle of the Assay: This assay employs the quantitative sandwich enzyme immunoassay technique. Antibody specific for MCCC1 has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and any MCCC1 present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for MCCC1 is added to the wells. After washing, avidin 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 MCCC1 bound in the initial step. The color development is stopped and the intensity of the color is measured.

80,473 Da

methylcrotonoyl-CoA carboxylase 1 (alpha)

methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial; MCCase subunit alpha; 3-methylcrotonyl-CoA carboxylase 1; methylcrotonoyl-Coenzyme A carboxylase 1 (alpha); 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha; 3-methylcrotonyl-CoA carboxylase biotin-containing subunit

Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial

MCCA; MCCase subunit alpha??[Similar Products]

MCCA_HUMAN

This gene encodes the large 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. [provided by RefSeq, Jul 2008]

MCCC1: Defects in MCCC1 are the cause of methylcrotonoyl-CoA carboxylase 1 deficiency (MCC1D). An autosomal recessive disorder of leucine catabolism. The phenotype is variable, ranging from neonatal onset with severe neurological involvement to asymptomatic *****s. 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.

Protein type: EC 6.4.1.4; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Mitochondrial; Ligase

Chromosomal Location of Human Ortholog: 3q27

Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial inner membrane; cytosol

Molecular Function: protein binding; methylcrotonoyl-CoA carboxylase activity; metal ion binding; biotin carboxylase activity; biotin binding; ATP binding

Biological Process: vitamin metabolic process; leucine catabolic process; branched chain family amino acid catabolic process; water-soluble vitamin metabolic process; biotin metabolic process

Disease: 3-methylcrotonyl-coa Carboxylase 1 Deficiency

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