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亨廷丁外子1与HEAT重复结构之间的相互作用由嵌合模型蛋白质探测到

Hong Zhang^1,2,3, Si Wu^1,2, Laura S Itzhaki⁴

¹National Laboratory of Biomacromolecules, CAS Center for Excellence in Biomacromolecules, Institute of Biophysics, Chinese Academy of Sciences, Beijing, China.

Protein science : a publication of the Protein Society

|October 19, 2023

概括

亨廷顿病 (HD) 涉及亨廷丁 (HTT) 蛋白质聚合. 这项研究探讨了HTT外子1与其HEAT重复结构的相互作用,揭示了对HD病变发生的洞察力.

科学领域:

神经科学是一个神经科学.
分子生物学分子生物学
蛋白质生物化学蛋白质生物化学

背景情况:

亨廷顿病 (HD) 与聚合亨廷丁 (HTT) 蛋白与扩展的多重氨酸 (polyQ) 重复在第1个外体中有关.
HTT的大小阻碍了结构研究,导致专注于外型1区域.
在HTT C端包含HEAT重复,对于细胞功能至关重要.

研究的目的:

为了研究HTT外子1区域及其HEAT重复结构之间的相互作用.
了解这种相互作用如何影响HD背景下的蛋白质稳定性,灵活性和纤维细胞形成.

主要方法:

构建结合HTT外子1和HEAT重复蛋白PR65/A的嵌合蛋白质.
分析HTT外基1对HEAT重复结构和灵活性的影响.
在具有不同多Q长度的嵌合蛋白中评估粉样纤维的形成.

主要成果:

HTT exon 1稍微破坏稳定,并增加了下游HEAT重复结构的结构灵活性.
多Q长度 (野生型与病理型) 并没有改变HTT外子1和HEAT重复之间的相互作用.
热反复的C端融合调节了病理性HTT外子1的纤维结构和形成动力学.

结论:

HTT外子1和HEAT重复之间的相互作用与正常的HTT功能和HD病原体兼容.
这项研究为进一步研究HTT结构-功能关系和HD机制提供了一个模型.

关键词:

热的重复重复一次.氨基化物氨基化物亨丁丁的前世纪第1部分多QQ多QQ多QQ是什么意思蛋白质折叠蛋白质的折叠

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