Ethylmalonyl-CoA Decarboxylase, a New Enzyme Involved in Metabolite Proofreading
Ethylmalonyl-CoA Decarboxylase, a New Enzyme Involved in Metabolite Proofreading
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DOI：
10.1074/jbc.m111.281527
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发表时间：
2011-12-16
影响因子：
4.8
通讯作者：
Van Schaftingen, Emile
中科院分区：
文献类型：
--
作者：
Linster, Carole L.;Noel, Gaetane;Van Schaftingen, Emile
背景资料：乙酰辅酶A和丙酰辅酶A羧化酶缓慢地形成乙基丙二酰辅酶A作为副活性。结果：在哺乳动物组织中鉴定出一种新的酶--乙基丙二酰辅酶A脱羧酶（ethylmalonyl-CoA decarboxylase），该酶由ECHDC 1基因编码。结论：乙基丙二酰辅酶A脱羧酶可能是一种新的“代谢物校正”酶，意义：它的缺乏可能导致乙基丙二酸尿症。
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.