期刊
JOURNAL OF BIOLOGICAL CHEMISTRY
卷 286, 期 50, 页码 42992-43003出版社
AMER SOC BIOCHEMISTRY MOLECULAR BIOLOGY INC
DOI: 10.1074/jbc.M111.281527
关键词
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资金
- Fonds de la Recherche Scientifique (FRS-FNRS)
- European Union [276814]
- Welbio (Walloon region)
Background: Acetyl-CoA and propionyl-CoA carboxylases slowly form ethylmalonyl-CoA as a side activity. Results: Anew enzyme, ethylmalonyl-CoA decarboxylase, was identified in mammalian tissues and shown to be encoded by the ECHDC1 gene. Knocking down this gene increased the formation of ethylmalonate from butyrate in cultured cells. Conclusion: Ethylmalonyl-CoA decarboxylase may be a new metabolite proofreading enzyme. Significance: Its deficiency may cause ethylmalonic aciduria.
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