Rabbit Anti-MCEE (Methylmalonyl-CoA Epimerase, Mitochondrial, DL-methylmalonyl-CoA Racemase, GLOD2)
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.
Immunogen
Full length human MCEE, aa1-176 (NP_115990.2).
Form
Supplied as a liquid in PBS, pH 7.2.
Purity
Purified by Protein A affinity chromatography.
Specificity
Recognizes human MCEE.
