Abbreviation: MCEE
Target Synonym: Methylmalonyl-CoA epimerase;mitochondrial;DL-methylmalonyl-CoA racemase
Target Species: Human
Expression Host: HEK293 Cells
Fusion Tag: C-His
UNIProt ID: Q96PE7
Background: Methylmalonyl-CoA epimerase, mitochondrial(MCEE)is an enzyme which belongs to the glyoxalase I family. It converts (S)-methylmalonyl-CoA to the (R) form, catalyses the following chemical reaction: (R)-methylmalonyl-CoA (S)-methylmalonyl-CoA. It plays an important role in the catabolism of fatty acids with odd-length carbon chains. This 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.
Sequence: Gln37-Ala176
Purity: > 95 % as determined by reducing SDS-PAGE.
Formulation: Lyophilized from sterile PBS, pH 7.4., 5% trehalose, 5% mannitol, 0.01% tween-80.
Normally 5 % - 8 % trehalose, mannitol and 0.01% Tween80 are added as protectants before lyophilization.
Please refer to the specific buffer information in the printed manual.
Endotoxin: < 1.0 EU per ug of the protein as determined by the LAL method.