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.

Dettagli prodotto

• Codice: PDMH100013_100μg
• Marca: Elabscience
• Specie target: Human
• Host: HEK293 Cells
• Confezionamento: Lyophilized from sterile PBS, pH 7.4., 5% trehalose, 5% mannitol, 0.01% Tween 80.<br/>Normally 5%-8% trehalose, mannitol and 0.01% Tween 80 are added as protectants before lyophilization.<br/>Please refer to the specific buffer information in the printed manual.
• Link: Apri link
• Stoccaggio: Generally, lyophilized proteins are stable for up to 12 months when stored at -20 to -80℃. Reconstituted protein solution can be stored at 4-8℃ for 2-7 days. Aliquots of reconstituted samples are stable at < -20℃ for 3 months.
• Simbolo target: MCEE
• Ig target: DL-methylmalonyl-CoA racemase;Methylmalonyl-CoA epimerase;mitochondrial