Human methylcrotonoyl-Coenzyme A carboxylase 1 (alpha) ELISA Kit | MCCC1 elisa kit

Human Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial, MCCC1 ELISA Kit

Cat. Num. AAA916524

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

• Reactivity: Human
• Specificity: This assay has high sensitivity and excellent specificity for detection of mouse MBP. No significant cross-reactivity or interference between mouse MBP and analogues was observed.
• Sequence Length: 725

• Samples: Serum, Plasma, Tissue Homogenates
• Assay Type: Quantitative Sandwich
• Detection Range: 18.75 pg/ml-1200 pg/ml
• Sensitivity: 4.69 pg/ml.
• Intra-assay Precision: Intra-assay Precision (Precision within an assay): CV%<8%. Three samples of known concentration were tested twenty times on one plate to assess
• Inter-assay Precision: Inter-assay Precision (Precision between assays): CV%<10%. Three samples of known concentration were tested in twenty assays to assess.
• Preparation and Storage: Unopened test kits should be stored at 2 to 8 degree C upon receipt. Please refer to pdf manual for further storage instructions.

Typical Testing Data/Standard Curve (for reference only)

Related Product Information for MCCC1 elisa kit

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

NCBI and Uniprot Product Information

• NCBI GI #: 116805327
• NCBI GeneID: 56922
• NCBI Accession #: NP_064551.3
• NCBI GenBank Nucleotide #: NM_020166.4
• UniProt Accession #: Q96RQ3; Q59ES4; Q9H959; Q9NS97
• Molecular Weight: 80,473 Da
• NCBI Official Full Name: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1
• NCBI Official Synonym Full Names: methylcrotonoyl-CoA carboxylase 1 (alpha)
• NCBI Official Symbol: MCCC1
• NCBI Official Synonym Symbols: MCCA; MCC-B
• NCBI Protein Information: 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
• UniProt Protein Name: Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
• UniProt Gene Name: MCCC1
• UniProt Synonym Gene Names: MCCA; MCCase subunit alpha
• UniProt Entry Name: MCCA_HUMAN

NCBI Description

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]

Uniprot Description

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 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.

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