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首页> 外文期刊>Proceedings of the National Academy of Sciences of the United States of America >Deficiency of a protein-repair enzyme results in the accumulation of altered proteins, retardation of growth, and fatal seizures in mice
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Deficiency of a protein-repair enzyme results in the accumulation of altered proteins, retardation of growth, and fatal seizures in mice

机译:蛋白质修复酶的缺乏会导致改变的蛋白质积聚,生长迟缓和致命性癫痫发作

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摘要

L-Asparaginyl and L-aspartyl residues in proteins are subject to spontaneous degradation reactions that generate isomerized and racemized aspartyl derivatives. Proteins containing L-isoaspartyl and D-aspartyl residues can have altered structures and diminished biological activity. These residues are recognized by a highly conserved cytosolic enzyme, the protein L-isoaspartate(D-aspartate) O-methyl- transferase (EC 2.1.1.77).
机译:蛋白质中的L-天冬酰胺基和L-天冬氨酰残基会发生自发降解反应,产生异构化和外消旋的天冬氨酰衍生物。含有L-异天冬氨酰和D-天冬氨酰残基的蛋白质可能具有改变的结构并降低了生物活性。这些残基被高度保守的胞质酶L-异天冬氨酸(D-天冬氨酸)O-甲基转移酶(EC 2.1.1.77)识别。

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