MCEE (Methylmalonyl-CoA Epimerase, Mitochondrial, DL-methylmalonyl-CoA Racemase, GLOD2)

Size

£760.00

SKU:: 129487
Application:: WB
Purity:: Purified by Protein A affinity chromatography.
Storage Conditions:: -20°C
Supplier:: United States Biological
Host:: Rabbit
Immunogen:: Full length human MCEE, aa1-176 (NP_115990.2).
Format:: Supplied as a liquid in PBS, pH 7.2.
Extra Details:: MCEE catalyzes the interconversion of D- and L-methylmalonyl-CoA during the degradation of branched chainaas, odd chain-length fatty acids, and other metabolites. This protein deficiency is an autosomal recessive inborn error ofaa 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. Applications: Suitable for use in Western Blot. Other applications not tested. Recommended Dilution: Optimal dilutions to be determined by the researcher. AA Sequence: MARVLKAAAANAVGLFSRLQAPIPTVRASSTSQPLDQVTGSVWNLGRLNHVAIAVPDLEKAAAFYKNILGAQVSEAVPLPEHGVSVVFVNLGNTKMELLHPLGLDSPIAGFLQKNKAGGMHHICIEVDNINAAVMDLKKKKIRSLSEEVKIGAHGKPVIFLHPKDCGGVLVELEQA Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.