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首页> 外文期刊>Proceedings of the National Academy of Sciences of the United States of America >Transglutaminase aggregates huntingtin into nonamyloidogenic polymers, and its enzymatic activity increases in Huntington's disease brain nuclei
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Transglutaminase aggregates huntingtin into nonamyloidogenic polymers, and its enzymatic activity increases in Huntington's disease brain nuclei

机译：转谷氨酰胺酶将亨廷顿蛋白聚集为非淀粉样生成物，其酶活性在亨廷顿氏病脑核中增加

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摘要

The protein huntingtin (htt), aggregated in neuronal nuclear inclusions, is pathognomonic of Hunting- ton's disease (HD). Constructs, translated in vitro from the N terminus of htt, containing either polyQ23 from a normal individual, or polyQ41 or polyQ67 from an HD patient, were all soluble. Transglutaminase (TGase) crosslinked these pro- teins, and the aggregations did not have the staining proper- ties of amyloid. More TGase-catalyzed aggregates formed when the polyglutamine domain of htt exceeded the pathologic threshold of polyQ36. Furthermore, shorter htt constructs, containing 135 aa or fewer, formed more aggregates than did larger htt constructs. TGase activity in the HD brain was increascd compared with the control, with notable increases in cell nuclei. The increased TGase activity was brain specific. In lymphoblastoid cells from HD patients, TGase activity was decreased. TGase-mediated crosslinking of htt may be in- volved in the formation of the nonamyloidogenic nuclear inclusions found in the HD brain. The staining properties of nuclear inclusions in tbe HD brain revealed that they were not amyloid.
机译：亨廷顿病（HD）的病理学特征是聚集在神经元核内含物中的亨廷顿蛋白（htt）。从htt N末端体外翻译的构建体均可溶，其中包含正常人的polyQ23或HD患者的polyQ41或polyQ67。转谷氨酰胺酶（TGase）使这些蛋白质交联，聚集体没有淀粉样蛋白的染色特性。当htt的聚谷氨酰胺结构域超过polyQ36的病理阈值时，会形成更多的TGase催化的聚集体。此外，与较大的htt构建体相比，包含135个氨基酸或更少氨基酸的较短的htt构建体形成更多的聚集体。与对照组相比，HD脑中的TGase活性增加，细胞核显着增加。 TGase活性增加是脑特异性的。在HD患者的淋巴母细胞中，TGase活性降低。 TGase介导的htt交联可能涉及HD脑中非淀粉样核包裹体的形成。高清大脑中核内含物的染色特性表明它们不是淀粉样蛋白。

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