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Evidence for a role for transglutaminase in Huntington's disease and the potential therapeutic implications.

机译:谷氨酰胺转移酶在亨廷顿舞蹈病中的作用及其潜在的治疗意义的证据。

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摘要

Transglutaminase (TGase) activity is increased in affected regions of brains from patients with Huntington's disease (HD). TGase activity is particularly elevated in the nucleus compared with the cytoplasm from these brains. Gamma-glutaminyl-lysyl cross-links have been detected in nuclear inclusions in HD brain, indicating that TGase may play a prominent role in the aggregation of huntingtin (htt). Attempts to ameliorate experimental disease, via inhibition of TGase in transgenic models of HD in mice, are under investigation.
机译:患有亨廷顿舞蹈病(HD)的患者的大脑受影响区域的转谷氨酰胺酶(TGase)活性增加。与来自这些大脑的细胞质相比,TGase活性在细胞核中特别提高。在高清大脑的核内含物中已检测到γ-谷氨酰胺基-赖氨酰交联,这表明TGase可能在亨廷顿蛋白(htt)的聚集中发挥重要作用。正在尝试通过抑制小鼠HD的转基因模型中的TGase来改善实验疾病。

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