MCEE recombinant protein

Recombinant Human MCEE Protein

Cat. Num. AAA8249436

• Gene Names: MCEE; GLOD2
• Reactivity: Human
• Applications: ELISA, Western Blot, SDS-Page
• Purity: Greater than 95% as determined by reducing SDS-PAGE.
• Synonyms: MCEE; Recombinant Human MCEE Protein; Methylmalonyl-CoA epimerase mitochondrial; DL-methylmalonyl-CoA racemase; MCEE recombinant protein

• Host: Human cells
• Reactivity: Human
• Purity/Purification: Greater than 95% as determined by reducing SDS-PAGE.
• Form/Format: Supplied as a 0.2 muM filtered solution of 20mM Tris-HCl, 150mM NaCl, 1mM DTT, 10% Glycerol, pH 7.5.
• Sequence: QVTGSVWNLG RLNHVAIAVP DLEKAAAFYK NILGAQVSEA VPLPEHGVSV VFVNLGNTKM ELLHPLGLDS PIAGFLQKNK AGGMHHICIE VDNINAAVMD LKKKKIRSLS EEVKIGAHGK PVIFLHPKDC GGVLVELEQA LDHHHHHH
• Sequence Length: 176

• Applicable Applications for MCEE recombinant protein: ELISA (EIA),Western Blot (WB), SDS-PAGE, Mass Spectrometry (MS)
• Endotoxin: Less than 0.1 ng/mug (1 IEU/mug) as determined by LAL test.
• Directions for Use: Always centrifuge tubes before opening. Do not mix by vortex or pipetting. It is not recommended to reconstitute to a concentration less than 100 mug/ml. Dissolve the lyophilized protein in 1X PBS. Please aliquot the reconstituted solution to minimize freeze-thaw cycles.
• Preparation and Storage: Lyophilized protein should be stored at -20 degree C, though stable at room temperature for 3 weeks. Reconstituted protein solution can be stored at 2-8 degree C for 2-7 days. Aliquots of reconstituted samples are stable at -20 degree C for 3 months.

Related Product Information for MCEE recombinant protein

Recombinant Human MCEE Protein is produced by our mammalian expression system and the target gene encoding Gln37-Ala176 is expressed with a 6His tag at the C-terminus.

NCBI and Uniprot Product Information

• NCBI GI #: 188035928
• NCBI GeneID: 84693
• NCBI Accession #: NP_115990.3
• NCBI GenBank Nucleotide #: NM_032601.3
• UniProt Accession #: Q96PE7; Q53TP1; Q8WW63
• Molecular Weight: 18,749 Da
• NCBI Official Full Name: methylmalonyl-CoA epimerase, mitochondrial
• NCBI Official Synonym Full Names: methylmalonyl-CoA epimerase
• NCBI Official Symbol: MCEE
• NCBI Official Synonym Symbols: GLOD2
• NCBI Protein Information: methylmalonyl-CoA epimerase, mitochondrial
• UniProt Protein Name: Methylmalonyl-CoA epimerase, mitochondrial
• UniProt Gene Name: MCEE
• UniProt Entry Name: MCEE_HUMAN

NCBI Description

The product of this gene 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. Mutations in this gene result in methylmalonyl-CoA epimerase deficiency, which is presented as mild to moderate methylmalonic aciduria. [provided by RefSeq, Jul 2008]

Uniprot Description

MCEE: Defects in MCEE are a cause of methylmalonyl-CoA epimerase deficiency (MCEED); also known as methylmalonyl-CoA racemase deficiency or methylmalonic aciduria type 3. MCEE 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. Belongs to the glyoxalase I family.

Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - propanoate; EC 5.1.99.1; Isomerase; Mitochondrial

Chromosomal Location of Human Ortholog: 2p13.3

Cellular Component: mitochondrial matrix

Molecular Function: methylmalonyl-CoA epimerase activity; protein binding

Biological Process: L-methylmalonyl-CoA metabolic process; short-chain fatty acid catabolic process

Disease: Methylmalonyl-coa Epimerase Deficiency