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Gene Review

MCEE  -  methylmalonyl CoA epimerase

Homo sapiens

Synonyms: DL-methylmalonyl-CoA racemase, GLOD2, Methylmalonyl-CoA epimerase, mitochondrial
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Disease relevance of MCEE


High impact information on MCEE

  • In this report, we identify the human DL-methylmalonyl-CoA racemase gene by analyzing prokaryotic gene arrangements and extrapolating the information obtained to human genes by homology searches [3].
  • The known types of reactions that are catalyzed include isomerizations (glyoxalase I), epimerizations (methylmalonyl-CoA epimerase), oxidative cleavage of C-C bonds (extradiol dioxygenase), and nucleophilic substitutions (fosfomycin resistance proteins) [4].
  • The C. elegans mce-1 deletion mutant demonstrates for the first time that a lesion at the epimerase step of methylmalonyl-CoA metabolism can functionally impair flux through the methylmalonyl-CoA mutase pathway and suggests that malfunction of MCEE may cause methylmalonic acidemia in humans [5].
  • To assess the impact of isolated MCE deficiency in cultured cells, HeLa cells were transfected with a selectable vector containing MCEE-specific small interfering RNA (siRNA) to suppress gene expression [6].
  • Propionyl-CoA and adenosylcobalamin metabolism in Caenorhabditis elegans: Evidence for a role of methylmalonyl-CoA epimerase in intermediary metabolism [5].

Associations of MCEE with chemical compounds


Analytical, diagnostic and therapeutic context of MCEE


  1. The effect of burn wound excision on measured energy expenditure and urinary nitrogen excretion. Ireton-Jones, C.S., Turner, W.W., Baxter, C.R. The Journal of trauma. (1987) [Pubmed]
  2. Crystal structure of methylmalonyl-coenzyme A epimerase from P. shermanii: a novel enzymatic function on an ancient metal binding scaffold. McCarthy, A.A., Baker, H.M., Shewry, S.C., Patchett, M.L., Baker, E.N. Structure (Camb.) (2001) [Pubmed]
  3. Identification of the human methylmalonyl-CoA racemase gene based on the analysis of prokaryotic gene arrangements. Implications for decoding the human genome. Bobik, T.A., Rasche, M.E. J. Biol. Chem. (2001) [Pubmed]
  4. Mechanistic diversity in a metalloenzyme superfamily. Armstrong, R.N. Biochemistry (2000) [Pubmed]
  5. Propionyl-CoA and adenosylcobalamin metabolism in Caenorhabditis elegans: Evidence for a role of methylmalonyl-CoA epimerase in intermediary metabolism. Chandler, R.J., Aswani, V., Tsai, M.S., Falk, M., Wehrli, N., Stabler, S., Allen, R., Sedensky, M., Kazazian, H.H., Venditti, C.P. Mol. Genet. Metab. (2006) [Pubmed]
  6. Homozygous nonsense mutation in the MCEE gene and siRNA suppression of methylmalonyl-CoA epimerase expression: a novel cause of mild methylmalonic aciduria. Dobson, C.M., Gradinger, A., Longo, N., Wu, X., Leclerc, D., Lerner-Ellis, J., Lemieux, M., Belair, C., Watkins, D., Rosenblatt, D.S., Gravel, R.A. Mol. Genet. Metab. (2006) [Pubmed]
  7. On the mechanism of action of methylmalonyl-CoA mutase. Change of the steric course on isotope substitution. Wölfle, K., Michenfelder, M., König, A., Hull, W.E., Rétey, J. Eur. J. Biochem. (1986) [Pubmed]
  8. Isolation and characterization of DL-methylmalonyl-coenzyme A racemase from rat liver. Stabler, S.P., Marcell, P.D., Allen, R.H. Arch. Biochem. Biophys. (1985) [Pubmed]
  9. HPLC assay for methylmalonyl-CoA epimerase. Bobik, T.A., Rasche, M.E. Analytical and bioanalytical chemistry. (2003) [Pubmed]
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