Recombinant human MCEE protein

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.

Product Specifications

Product Name Alternative

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

Expression System

E.coli

Antigen Species

Human

Tag

His-Tag

Applications

SDS-PAGE

Concentration

1 mg/mL (determined by Bradford assay)

Purity

> 90% by SDS-PAGE

Molecular Weight

17.3 kDa (161aa) confirmed by MALDI-TOF

Additionnal Information

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

References & Citations

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

Storage Conditions

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.

Formulation

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

Scientific Category

Enzymes & Proteases

NCBI Accession Number

NP_115990

Uniprot Accession Number

Q96PE7

Species

Human

AA Sequence

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