Recombinant human MCEE protein
MCEE 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. This protein 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. Recombinant human MCEE protein, fused to His-tag at N-terminus, was expressed in E. coli and purified by using conventional chromatography techniques.
Product Specifications
Product Name Alternative
Methylmalonyl CoA epimerase, DL methylmalonyl CoA racemase, EC 5.1.99.1, GLOD2, Glyoxalase domain containing 2
Expression System
E.coli
Antigen Species
Human
Tag
His-Tag
Applications
SDS-PAGE
Concentration
1 mg/mL (determined by Bradford assay)
Purity
> 90% by SDS-PAGE
Molecular Weight
17.3 kDa (161aa) confirmed by MALDI-TOF
Additionnal Information
References & Citations
Yang GE., et al. (2009) Appl Biochem Biotechnol. 152 (3) :353-65.; ; Gradinger AB., et al. (2007) Hum Mutat. 28 (10) :1045.
Storage Conditions
Can be stored at 2°C to 8°C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.
Formulation
Liquid in. 20 mM Tris-HCl buffer (pH 8.0) containing 0.2M NaCl, 1mM DTT, 0.1mM PMSF, 10% glycerol
Scientific Category
Enzymes & Proteases
NCBI Accession Number
NP_115990
Uniprot Accession Number
Q96PE7
Species
Human
AA Sequence
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