Mouse Methylmalonyl-CoA epimerase ELISA Kit | MCEE elisa kit

Mouse Methylmalonyl-CoA epimerase, mitochondrial (MCEE) ELISA Kit

Cat. Num. AAA283022

• Gene Names: Mcee; 1110007A04Rik
• Reactivity: Mouse
• Synonyms: Methylmalonyl-CoA epimerase; Mouse Methylmalonyl-CoA epimerase; mitochondrial (MCEE) ELISA Kit; MCEE elisa kit

• Reactivity: Mouse
• Specificity: This assay has high sensitivity and excellent specificity for detection of Mouse MCEE. No significant cross-reactivity or interference between Mouse MCEE and analogues was observed.
• Sequence Length: 131

• Samples: Serum, Plasma, Other biological fluids
• Assay Type: Sandwich
• Sample Volume: 1-200 uL
• Precision: Intra-assay Precision (Precision within an assay); Three samples of known concentration were tested twenty times on one plate to assess intra-assay precision.; Inter-assay Precision (Precision between assays); Three samples of known concentration were tested in forty separate assays to assess inter-assay precision.; CV (%) = SD/meanX100; Intra-Assay: CV; Inter-Assay: CV
• Detection Wavelength: 450 nm
• Preparation and Storage: Store at 2-8 degree C.

Related Product Information for MCEE elisa kit

Methylmalonyl CoA Epimerase catalyzes the interconversion of D- and L-methylmalonyl-CoA during the degradation of branched chain amino acids. odd chain-length fatty acids, and other metabolites. Mutations in this gene result in methylmalonyl-CoA epimerase deficiency, which is presented as mild to moderate methylmalonic aciduria.Bikker et al. (2006) reported a 16-year-old female patient with persistent moderate methylmalonic aciduria. In vitro biochemical findings that comprised a decreased propionate incorporation into macromolecules in cultured fibroblasts and a fully normal activity of methylmalonyl-CoA mutase in the same cells were fully explained by deficiency of methylmalonyl-CoA epimerase.

Principle of the Assay: This assay employs a two-site sandwich ELISA to quantitate MCEE in samples. An antibody specific for MCEE has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and anyMCEE present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for MCEE 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 MCEE 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 #: 58037329
• NCBI GeneID: 73724
• NCBI Accession #: NP_082902.1
• NCBI GenBank Nucleotide #: NM_028626.2
• UniProt Accession #: Q9D1I5
• Molecular Weight: 19,017 Da
• NCBI Official Full Name: methylmalonyl-CoA epimerase, mitochondrial isoform 1
• NCBI Official Synonym Full Names: methylmalonyl CoA epimerase
• NCBI Official Symbol: Mcee
• NCBI Official Synonym Symbols: 1110007A04Rik
• NCBI Protein Information: methylmalonyl-CoA epimerase, mitochondrial
• UniProt Protein Name: Methylmalonyl-CoA epimerase, mitochondrial
• Protein Family: Methylmalonyl-CoA epimerase
• UniProt Gene Name: Mcee

Uniprot Description

MCEE: Defects in MCEE are a cause of methylmalonyl-CoA epimerase deficiency (MCEED); also known as methylmalonyl-CoA racemase deficiency or methylmalonic aciduria type 3. MCEE deficiency is an autosomal recessive inborn error of amino acid metabolism, involving valine, threonine, isoleucine and methionine. This organic aciduria may present in the neonatal period with life-threatening metabolic acidosis, hyperammonemia, feeding difficulties, pancytopenia and coma. Belongs to the glyoxalase I family.

Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - propanoate; EC 5.1.99.1; Isomerase; Mitochondrial

Chromosomal Location of Human Ortholog: 7|7 C

Cellular Component: mitochondrion

Molecular Function: isomerase activity; metal ion binding; methylmalonyl-CoA epimerase activity

Biological Process: L-methylmalonyl-CoA metabolic process