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1
Huntingtin aggregation monitored by dynamic light scattering.通过动态光散射监测亨廷顿蛋白聚集。
Proc Natl Acad Sci U S A. 1998 May 26;95(11):6118-21. doi: 10.1073/pnas.95.11.6118.
2
Aggregation of truncated GST-HD exon 1 fusion proteins containing normal range and expanded glutamine repeats.包含正常范围和扩展谷氨酰胺重复序列的截短型GST-HD外显子1融合蛋白的聚集。
Philos Trans R Soc Lond B Biol Sci. 1999 Jun 29;354(1386):991-4. doi: 10.1098/rstb.1999.0450.
3
Huntingtin's WW domain partners in Huntington's disease post-mortem brain fulfill genetic criteria for direct involvement in Huntington's disease pathogenesis.亨廷顿舞蹈病尸检大脑中亨廷顿蛋白的WW结构域结合蛋白符合直接参与亨廷顿舞蹈病发病机制的遗传学标准。
Hum Mol Genet. 2000 Sep 1;9(14):2175-82. doi: 10.1093/hmg/9.14.2175.
4
Identification of novel potentially toxic oligomers formed in vitro from mammalian-derived expanded huntingtin exon-1 protein.鉴定体外来源于哺乳动物衍生的扩展 huntingtin 外显子-1 蛋白的新型潜在毒性寡聚物。
J Biol Chem. 2012 May 4;287(19):16017-28. doi: 10.1074/jbc.M111.252577. Epub 2012 Mar 20.
5
Membrane filter assay for detection of amyloid-like polyglutamine-containing protein aggregates.用于检测含聚谷氨酰胺的淀粉样蛋白样蛋白聚集体的膜过滤分析
Methods Enzymol. 1999;309:375-86. doi: 10.1016/s0076-6879(99)09026-6.
6
Nucleation of huntingtin aggregation in cells.细胞中亨廷顿蛋白聚集的成核作用。
Nat Chem Biol. 2006 Jun;2(6):297-8. doi: 10.1038/nchembio0606-297.
7
Huntingtin aggregates ask to be eaten.亨廷顿蛋白聚集体渴望被清除。
Nat Genet. 2004 Jun;36(6):553-4. doi: 10.1038/ng0604-553.
8
Mutant huntingtin forms in vivo complexes with distinct context-dependent conformations of the polyglutamine segment.突变型亨廷顿蛋白在体内与多聚谷氨酰胺片段不同的上下文依赖构象形成复合物。
Neurobiol Dis. 1999 Oct;6(5):364-75. doi: 10.1006/nbdi.1999.0260.
9
Rapid aggregate formation of the huntingtin N-terminal fragment carrying an expanded polyglutamine tract.携带扩展型聚谷氨酰胺序列的亨廷顿蛋白N端片段的快速聚集形成。
Biochem Biophys Res Commun. 1999 Mar 16;256(2):361-6. doi: 10.1006/bbrc.1999.0337.
10
Green tea (-)-epigallocatechin-gallate modulates early events in huntingtin misfolding and reduces toxicity in Huntington's disease models.绿茶（-）-表没食子儿茶素-3-没食子酸酯调节亨廷顿蛋白错误折叠的早期事件并降低亨廷顿舞蹈病模型中的毒性。
Hum Mol Genet. 2006 Sep 15;15(18):2743-51. doi: 10.1093/hmg/ddl210. Epub 2006 Aug 7.

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1
Investigating the effects of N-terminal acetylation on KFE8 self-assembly with 2D IR spectroscopy.采用二维红外光谱法研究 N 端乙酰化对 KFE8 自组装的影响。
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2
Challenges Associated With the Formation of Recombinant Protein Inclusion Bodies in and Strategies to Address Them for Industrial Applications.重组蛋白包涵体形成所涉及的挑战及其在工业应用中的应对策略。
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Congo Red and amyloids: history and relationship.刚果红与淀粉样物质：历史与关系。
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Generation of Native, Untagged Huntingtin Exon1 Monomer and Fibrils Using a SUMO Fusion Strategy.使用SUMO融合策略生成天然、无标签的亨廷顿蛋白外显子1单体和原纤维。
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5
Activity of Zn and Mg phthalocyanines and porphyrazines in amyloid aggregation of insulin.锌和镁酞菁及卟啉嗪在胰岛素淀粉样聚集过程中的活性
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6
PIN1 Modulates Huntingtin Levels and Aggregate Accumulation: An Model.PIN1调节亨廷顿蛋白水平和聚集体积累：一种模型。
Front Cell Neurosci. 2017 May 8;11:121. doi: 10.3389/fncel.2017.00121. eCollection 2017.
7
Identification of hepta-histidine as a candidate drug for Huntington's disease by in silico-in vitro- in vivo-integrated screens of chemical libraries.通过化学文库的计算机模拟-体外-体内综合筛选鉴定七聚组氨酸作为亨廷顿舞蹈病的候选药物。
Sci Rep. 2016 Sep 22;6:33861. doi: 10.1038/srep33861.
8
An Intein-based Strategy for the Production of Tag-free Huntingtin Exon 1 Proteins Enables New Insights into the Polyglutamine Dependence of Httex1 Aggregation and Fibril Formation.一种基于内含肽的策略用于生产无标签亨廷顿蛋白外显子1蛋白，这为深入了解Httex1聚集和纤维形成的多聚谷氨酰胺依赖性提供了新的见解。
J Biol Chem. 2016 Jun 3;291(23):12074-86. doi: 10.1074/jbc.M116.713982. Epub 2016 Mar 21.
9
Single-molecule analyses of the dynamics of heat shock protein 104 (Hsp104) and protein aggregates.热休克蛋白104（Hsp104）与蛋白质聚集体动力学的单分子分析
J Biol Chem. 2015 Mar 20;290(12):7833-40. doi: 10.1074/jbc.M114.620427. Epub 2015 Jan 29.
10
Analyzing subvisible particles in protein drug products: a comparison of dynamic light scattering (DLS) and resonant mass measurement (RMM).分析蛋白药物制品中的亚可见颗粒：动态光散射（DLS）和共振质量测量（RMM）的比较。
AAPS J. 2014 May;16(3):440-51. doi: 10.1208/s12248-014-9579-6. Epub 2014 Feb 26.

本文引用的文献

1
Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo.亨廷顿蛋白编码的多聚谷氨酰胺扩展在体外和体内形成淀粉样蛋白聚集体。
Cell. 1997 Aug 8;90(3):549-58. doi: 10.1016/s0092-8674(00)80514-0.
2
Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation.神经元核内包涵体的形成是亨廷顿舞蹈症突变转基因小鼠神经功能障碍的基础。
Cell. 1997 Aug 8;90(3):537-48. doi: 10.1016/s0092-8674(00)80513-9.
3
Models of amyloid seeding in Alzheimer's disease and scrapie: mechanistic truths and physiological consequences of the time-dependent solubility of amyloid proteins.
Annu Rev Biochem. 1997;66:385-407. doi: 10.1146/annurev.biochem.66.1.385.
4
Kinetic theory of fibrillogenesis of amyloid beta-protein.淀粉样β蛋白原纤维形成的动力学理论。
Proc Natl Acad Sci U S A. 1997 Jul 22;94(15):7942-7. doi: 10.1073/pnas.94.15.7942.
5
Identification of an HD patient with a (CAG)180 repeat expansion and the propagation of highly expanded CAG repeats in lambda phage.
Hum Genet. 1997 May;99(5):692-5. doi: 10.1007/s004390050432.
6
Glutamine repeats and inherited neurodegenerative diseases: molecular aspects.谷氨酰胺重复序列与遗传性神经退行性疾病：分子层面
Curr Opin Struct Biol. 1996 Dec;6(6):848-58. doi: 10.1016/s0959-440x(96)80016-9.
7
Phenotypic characterization of individuals with 30-40 CAG repeats in the Huntington disease (HD) gene reveals HD cases with 36 repeats and apparently normal elderly individuals with 36-39 repeats.亨廷顿舞蹈病（HD）基因中含有30 - 40个CAG重复序列的个体的表型特征显示，存在具有36个重复序列的HD病例以及具有36 - 39个重复序列的明显正常的老年人。
Am J Hum Genet. 1996 Jul;59(1):16-22.
8
On the nucleation and growth of amyloid beta-protein fibrils: detection of nuclei and quantitation of rate constants.关于β-淀粉样蛋白原纤维的成核与生长：核的检测及速率常数的定量分析
Proc Natl Acad Sci U S A. 1996 Feb 6;93(3):1125-9. doi: 10.1073/pnas.93.3.1125.
9
A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's Disease Collaborative Research Group.一个含有三核苷酸重复序列的新基因，该序列在亨廷顿病染色体上呈扩增且不稳定状态。亨廷顿病协作研究组。
Cell. 1993 Mar 26;72(6):971-83. doi: 10.1016/0092-8674(93)90585-e.
10
Light scattering analysis of fibril growth from the amino-terminal fragment beta(1-28) of beta-amyloid peptide.β-淀粉样肽氨基末端片段β(1 - 28) 原纤维生长的光散射分析
Biophys J. 1993 Dec;65(6):2383-95. doi: 10.1016/S0006-3495(93)81312-2.

通过动态光散射监测亨廷顿蛋白聚集。

Huntingtin aggregation monitored by dynamic light scattering.

作者信息

Georgalis Y, Starikov E B, Hollenbach B, Lurz R, Scherzinger E, Saenger W, Lehrach H, Wanker E E

机构信息

Institut für Kristallographie, Freie Universität Berlin, Takustr. 6, D-14195 Berlin, Germany.

出版信息

Proc Natl Acad Sci U S A. 1998 May 26;95(11):6118-21. doi: 10.1073/pnas.95.11.6118.

DOI:10.1073/pnas.95.11.6118
PMID:9600927
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC27595/
Abstract

An initial stage of fibrillogenesis in solutions of glutathione S-transferase-huntingtin (GST-HD) fusion proteins has been studied by using dynamic light scattering. Two GST-HD systems with poly-L-glutamine (polyGln) extensions of different lengths (20 and 51 residues) have been examined. For both systems, kinetics of z-average translation diffusion coefficients (Dapp) and their angular dependence have been obtained. Our data reveal that aggregation does occur in both GST-HD51 and GST-HD20 solutions, but that it is much more pronounced in the former. Thus, our approach provides a powerful tool for the quantitative assay of GST-HD fibrillogenesis in vitro.

摘要

利用动态光散射研究了谷胱甘肽S-转移酶-亨廷顿蛋白（GST-HD）融合蛋白溶液中纤维生成的初始阶段。研究了两种具有不同长度（20和51个残基）聚-L-谷氨酰胺（polyGln）延伸的GST-HD系统。对于这两种系统，均获得了z平均平移扩散系数（Dapp）的动力学及其角度依赖性。我们的数据表明，GST-HD51和GST-HD20溶液中均发生聚集，但前者更为明显。因此，我们的方法为体外定量测定GST-HD纤维生成提供了一个强大的工具。