Zernike Institute for Advanced Materials, University of Groningen, Nijenborgh 4, 9747 AG Groningen, The Netherlands.
Stratingh Institute for Chemistry, University of Groningen, Nijenborgh 7, 9747 AG Groningen, The Netherlands.
J Am Chem Soc. 2024 Jan 24;146(3):2062-2071. doi: 10.1021/jacs.3c11155. Epub 2024 Jan 16.
A family of neurodegenerative diseases, including Huntington's disease (HD) and spinocerebellar ataxias, are associated with an abnormal polyglutamine (polyQ) expansion in mutant proteins that become prone to form amyloid-like aggregates. Prior studies have suggested a key role for β-hairpin formation as a driver of nucleation and aggregation, but direct experimental studies have been challenging. Toward such research, we set out to enable spatiotemporal control over β-hairpin formation by the introduction of a photosensitive β-turn mimic in the polypeptide backbone, consisting of a newly designed azobenzene derivative. The reported derivative overcomes the limitations of prior approaches associated with poor photochemical properties and imperfect structural compatibility with the desired β-turn structure. A new azobenzene-based β-turn mimic was designed, synthesized, and found to display improved photochemical properties, both prior and after incorporation into the backbone of a polyQ polypeptide. The two isomers of the azobenzene-polyQ peptide showed different aggregate structures of the polyQ peptide fibrils, as demonstrated by electron microscopy and solid-state NMR (ssNMR). Notably, only peptides in which the β-turn structure was stabilized (azobenzene in the configuration) closely reproduced the spectral fingerprints of toxic, β-hairpin-containing fibrils formed by mutant huntingtin protein fragments implicated in HD. These approaches and findings will enable better deciphering of the roles of β-hairpin structures in protein aggregation processes in HD and other amyloid-related neurodegenerative diseases.
一类神经退行性疾病,包括亨廷顿病(HD)和脊髓小脑共济失调,与突变蛋白中异常的多聚谷氨酰胺(polyQ)扩展有关,这些突变蛋白容易形成类似淀粉样的聚集物。先前的研究表明β发夹结构的形成是核形成和聚集的关键驱动力,但直接的实验研究具有挑战性。为此,我们通过在多肽主链中引入光敏感的β-转角模拟物来实现β发夹形成的时空控制,该模拟物由新设计的偶氮苯衍生物组成。所报道的衍生物克服了先前方法的局限性,这些方法与较差的光化学性质以及与所需β-转角结构的不完全结构兼容性有关。设计、合成了一种新的基于偶氮苯的β-转角模拟物,并发现其在掺入聚 Q 多肽的主链前后都具有改善的光化学性质。两种偶氮苯-聚 Q 肽的异构体显示了聚 Q 肽原纤维的不同聚集结构,这通过电子显微镜和固态 NMR(ssNMR)得到证实。值得注意的是,只有稳定β-转角结构的肽(偶氮苯在 构象)才能紧密复制由与 HD 相关的突变亨廷顿蛋白片段形成的有毒β-发夹原纤维的光谱特征。这些方法和发现将能够更好地阐明β发夹结构在 HD 和其他与淀粉样蛋白相关的神经退行性疾病中的蛋白聚集过程中的作用。