Product Description for MCEE
MCEE.
Presentation: Purified
Presentation: Purified
Properties for MCEE
| Product Category | Proteins & Growth Factors |
|---|---|
| Quantity | 0.5 mg |
| Synonyms | DL-methylmalonyl-CoA racemase, mitochondrial Methylmalonyl-CoA epimerase |
| Presentation | Purified |
| Molecular weight | 17.3 kDa |
| Source | E. coli |
| Species (Protein) | Human |
| Shipping to | Worldwide |
| PDF datasheet | View Datasheet |
| Manufacturer | OriGene Technologies GmbH |
| Material safety datasheet | MSDS for Proteins (de) |
Datasheet Extract
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| Purity | > 90 % |
| Background | 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. |
| Concentration | 1.0 mg/ml (determined by Bradford assay) |
| General Readings | Yang GE., et al. (2009) Appl Biochem Biotechnol. 152(3):353-65. Gradinger AB., et al. (2007) Hum Mutat. 28(10):1045. |
| Storage | Store undiluted at 2-8°C for up to two weeks or (in aliquots) at -20°C or -70°C for longer. Avoid repeated freezing and thawing. Shelf life: one year from despatch. |
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