MCEE catalyzes the interconversion of D- and L-methylmalonyl-CoA during the degradation of branched chainaas, odd chain-length fatty acids, and other metabolites. This protein deficiency is an autosomal recessive inborn error ofaa 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.||Source:|Recombinant corresponding to aa37-176 of human MCEE protein, fused to His-tag at N-terminus, expressed in E.coli.||AA Sequence:|MGSSHHHHHH SSGLVPRGSH MQVTGSVWNL GRLNHVAIAV PDLEKAAAFY KNILGAQVSE AVPLPEHGVS VVFVNLGNTK MELLHPLGRD SPIAGFLQKN KAGGMHHICI EVDNINAAVM DLKKKKIRSL SEEVKIGAHG KPVIFLHPKD CGGVLVELEQ A||Molecular Weight: 17.3kD (161aa) confirmed by MALDI-TOF||Enzyme Activity:|Not determined. This product is recommended for use in applications that do not require a catalytically active form of the protein.||Storage and Stability:|May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for at least 6 months at -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

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