Purity: > 90% by SDS - PAGE Format: Liquid. In 20 mM Tris-HCl buffer (pH8.0) containing 0.2M NaCl, 1mM DTT, 0.1mM PMSF, 10% glycerol. 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.
Size
0.5mg, 100ug, 20ug
Concentration
n/a
Applications
SDS-PAGE
Other Names
Methylmalonyl CoA epimerase, DL methylmalonyl CoA racemase, EC 5.1.99.1, GLOD2, Glyoxalase domain containing 2