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Exploding the Repeat Length Paradigm while Exploring Amyloid Toxicity in Huntington’s Disease
Accounts of Chemical Research ( IF 18.3 ) Pub Date : 2020-09-25 , DOI: 10.1021/acs.accounts.0c00450 Ronald Wetzel 1
Accounts of Chemical Research ( IF 18.3 ) Pub Date : 2020-09-25 , DOI: 10.1021/acs.accounts.0c00450 Ronald Wetzel 1
Affiliation
Huntington’s disease (HD) is a progressive, familial neurodegenerative disease triggered by the expansion of a polyglutamine (polyQ) track in the protein huntingtin (htt). PolyQ sequences up to Q36 in htt are not known to be toxic, while polyQ lengths above Q36 almost invariably lead to increased disease risk and decreased ages of onset. The large number of physical states (monomers, dimers, tetramers, non-β oligomers, nanofibrils, and clustered amyloid fibrils) on the self-association landscape, with their overlapping kinetics of formation, have greatly complicated identification of the molecular species responsible for HD toxicity, drawing attention to the need for innovative approaches.
中文翻译:
在研究亨廷顿氏病的淀粉样蛋白毒性的同时探索重复长度范例
亨廷顿舞蹈病(HD)是一种渐进性家族性神经退行性疾病,由亨廷顿蛋白(htt)中的聚谷氨酰胺(polyQ)轨迹扩展引起。已知在htt中达到Q 36的PolyQ序列是有毒的,而高于Q 36的polyQ长度几乎总是导致疾病风险增加和发病年龄降低。自缔合态势上的大量物理状态(单体,二聚体,四聚体,非β低聚体,纳米原纤维和簇状淀粉样原纤维)及其形成动力学相互重叠,对导致HD的分子种类的识别非常复杂毒性,引起人们对创新方法需求的关注。
更新日期:2020-10-21
中文翻译:
在研究亨廷顿氏病的淀粉样蛋白毒性的同时探索重复长度范例
亨廷顿舞蹈病(HD)是一种渐进性家族性神经退行性疾病,由亨廷顿蛋白(htt)中的聚谷氨酰胺(polyQ)轨迹扩展引起。已知在htt中达到Q 36的PolyQ序列是有毒的,而高于Q 36的polyQ长度几乎总是导致疾病风险增加和发病年龄降低。自缔合态势上的大量物理状态(单体,二聚体,四聚体,非β低聚体,纳米原纤维和簇状淀粉样原纤维)及其形成动力学相互重叠,对导致HD的分子种类的识别非常复杂毒性,引起人们对创新方法需求的关注。