Recombinant humaan MCEE-eiwit

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. Recombinant human MCEE protein, fused to His-tag at N-terminus, was expressed in E. coli and purified by using conventional chromatography techniques.

Methylmalonyl CoA epimerase, DL methylmalonyl CoA racemase, EC 5.1.99.1, GLOD2, Glyoxalase domain containing 2

E.coli

Human

His-Tag

SDS-PAGE

1 mg/mL (determined by Bradford assay)

> 90% by SDS-PAGE

17.3 kDa (161aa) confirmed by MALDI-TOF

MCEE, Methylmalonyl CoA epimerase, DL methylmalonyl CoA racemase, EC 5.1.99.1, GLOD2, Glyoxalase domain containing 2, ATGP0752-10 µg, ATGP0752-20 µg, ATGP0752-50 µg, ATGP0752-100 µg, ATGP0752-250 µg, ATGP0752-500 µg, ATGP0752-1 mg, ATGP0752-10, ATGP0752-20, ATGP0752-50, ATGP0752-100, ATGP0752-250, ATGP0752-500, ATGP0752-1

Yang GE., et al. (2009) Appl Biochem Biotechnol. 152 (3) :353-65.; ; Gradinger AB., et al. (2007) Hum Mutat. 28 (10) :1045.

Can be stored at 2°C to 8°C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.

Liquid in. 20 mM Tris-HCl buffer (pH 8.0) containing 0.2M NaCl, 1mM DTT, 0.1mM PMSF, 10% glycerol

Enzymes & Proteases

NP_115990

Q96PE7

Human

MGSSHHHHHH SSGLVPRGSH MQVTGSVWNL GRLNHVAIAV PDLEKAAAFY KNILGAQVSE AVPLPEHGVS VVFVNLGNTK MELLHPLGRD SPIAGFLQKN KAGGMHHICI EVDNINAAVM DLKKKKIRSL SEEVKIGAHG KPVIFLHPKD CGGVLVELEQ A