Methylmalonyl CoA Epimerase (MCEE) (AA 37-176) protein (His tag)
Description
MCEE catalyzes the interconversion of D- and L-methylmalonyl-CoA throughout the degradation of branched chain AA, odd chain-length fatty acids, and other metabolites. MCEE protein deficiency is an autosomal recessive inborn error of AA metabolism, involving valine, threonine, isoleucine and methionine. This organic aciduria can appear in the neonatal period with life-threatening metabolic acidosis, hyperammonemia, feeding difficulties, pancytopenia and coma Synonyms: GLOD2, Methylmalonyl CoA Epimerase, Glyoxalase Domain Containing 2, DL-methylmalonyl-CoA Racemase