MCEE, Blocking Peptide

Methylmalonyl-CoA epimerase; mitochondrial; DL-methylmalonyl-CoA racemase; MCEE

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

Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Small volumes of MCEE blocking peptide 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.

18,749 Da

methylmalonyl-CoA epimerase

methylmalonyl-CoA epimerase, mitochondrial

Methylmalonyl-CoA epimerase, mitochondrial

MCEE_HUMAN

The product of this gene 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. [provided by RefSeq, Jul 2008]

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; Mitochondrial; Isomerase; EC 5.1.99.1

Chromosomal Location of Human Ortholog: 2p13.3

Cellular Component: mitochondrial matrix

Molecular Function: methylmalonyl-CoA epimerase activity; protein binding

Biological Process: L-methylmalonyl-CoA metabolic process; short-chain fatty acid catabolic process

Disease: Methylmalonyl-coa Epimerase Deficiency

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