Recombinant Human Methylmalonyl-CoA Epimerase/MCEE
Product name: | Recombinant Human Methylmalonyl-CoA Epimerase/MCEE |
Source: | Human Cells |
Purity: | Greater than 95% as determined by reducing SDS-PAGE. |
Buffer Formulation: | Supplied as a 0.2 μm filtered solution of 20mM TrisHCl,150mM NaCl,1mM DTT,10%Glycerol,pH7.5. |
Applications: | Applications:SDS-PAGE; WB; ELISA; IP. |
Storage: | Avoid repeated freeze/thaw cycles. Store at 2-8 oC for one month. Aliquot and store at -80 oC for 12 months. |
UOM: | 100ug/50ug/200ug/1mg/1g |
Source | Human Cells |
Description | Recombinant Human Methylmalonyl-CoA epimerase is produced by our Mammalian expression system and the target gene encoding Gln37-Ala176 is expressed with a 6His tag at the C-terminus. |
Names | Methylmalonyl-CoA epimerase, mitochondrial,DL-methylmalonyl-CoA racemase |
Accession # | Q96PE7 |
Formulation | Supplied as a 0.2 μm filtered solution of 20mM TrisHCl,150mM NaCl,1mM DTT,10%Glycerol,pH7.5. |
Shipping |
The product is shipped on dry ice/ice packs. |
Storage |
Store at < -20°C, stable for 6 months after receipt. Please minimize freeze-thaw cycles. |
Purity |
Greater than 95% as determined by reducing SDS-PAGE. |
Endotoxin | Less than 0.1 ng/µg (1 IEU/µg) as determined by LAL test. |
Amino Acid Sequence |
QVTGSVWNLGRLNHVAIAVPDLEKAAAFYKNILGAQVSEAVPLPEHGVSVVFVNLGNTKMELLHP LGLDSPIAGFLQKNKAGGMHHICIEVDNINAAVMDLKKKKIRSLSEEVKIGAHGKPVIFLHPKDC GGVLVELEQALDHHHHHH
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Background | Methylmalonyl-CoA epimerase, mitochondrial(MCEE)is an enzyme which belongs to the glyoxalase I family. It converts (S)-methylmalonyl-CoA to the (R) form, catalyses the following chemical reaction: (R)-methylmalonyl-CoA (S)-methylmalonyl-CoA. It plays an important role in the catabolism of fatty acids with odd-length carbon chains. This protein deficiency is an autosomal recessive inborn error of AA metabolism, involving valine, threonine, isoleucine and methionine. This organic aciduria can appear in the neonatal period with life-threatening metabolic acidosis, hyperammonemia, feeding difficulties, pancytopenia and coma. |