||Recombinant human MCEE protein, fused to His-tag at N-terminus, was expressed in E.coli and purified by using conventional chromatography techniques. MW = 17.3 kDa (161aa) confirmed by MALDI-TOF.
||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.
|Sequences Of Amino Acids:
||MGSSHHHHHH SSGLVPRGSH MQVTGSVWNL GRLNHVAIAV PDLEKAAAFY KNILGAQVSE AVPLPEHGVS VVFVNLGNTK MELLHPLGRD SPIAGFLQKN KAGGMHHICI EVDNINAAVM DLKKKKIRSL SEEVKIGAHG KPVIFLHPKD CGGVLVELEQ A
||> 90% by SDS – PAGE.
||Liquid. In 20 mM Tris-HCl buffer (pH8.0) containing 0.2M NaCl, 1mM DTT, 0.1mM PMSF, 10% glycerol.
||1 mg/ml (determined by Bradford assay).
||Can be stored at +4°C short term (1-2 weeks). For long term storage, aliquot and store at -20°C or -70°C. Avoid repeated freezing and thawing cycles.