A flexible polyglutamine hinge opens new doors for understanding huntingtin function.
作者信息
Giorgini Flaviano
机构信息
Department of Genetics, University of Leicester, Leicester LE1 7RH, United Kingdom.
出版信息
Proc Natl Acad Sci U S A. 2013 Sep 3;110(36):14516-7. doi: 10.1073/pnas.1313668110. Epub 2013 Aug 23.
Abstract
摘要
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A flexible polyglutamine hinge opens new doors for understanding huntingtin function.一个灵活的聚谷氨酰胺铰链为理解亨廷顿蛋白的功能打开了新的大门。
Proc Natl Acad Sci U S A. 2013 Sep 3;110(36):14516-7. doi: 10.1073/pnas.1313668110. Epub 2013 Aug 23.
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Expression of full-length polyglutamine-expanded Huntingtin disrupts growth factor receptor signaling in rat pheochromocytoma (PC12) cells.全长多聚谷氨酰胺扩展的亨廷顿蛋白的表达会破坏大鼠嗜铬细胞瘤（PC12）细胞中的生长因子受体信号传导。
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本文引用的文献
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Polyglutamine domain flexibility mediates the proximity between flanking sequences in huntingtin.多聚谷氨酰胺结构域的柔性调节亨廷顿蛋白侧翼序列的接近程度。
Proc Natl Acad Sci U S A. 2013 Sep 3;110(36):14610-5. doi: 10.1073/pnas.1301342110. Epub 2013 Jul 29.
2
Suppressing aberrant GluN3A expression rescues synaptic and behavioral impairments in Huntington's disease models.抑制异常的 GluN3A 表达可挽救亨廷顿病模型中的突触和行为损伤。
Nat Med. 2013 Aug;19(8):1030-8. doi: 10.1038/nm.3246. Epub 2013 Jul 14.
3
The interaction of polyglutamine peptides with lipid membranes is regulated by flanking sequences associated with huntingtin.多聚谷氨酰胺肽与脂膜的相互作用受与亨廷顿蛋白相关的侧翼序列调节。
J Biol Chem. 2013 May 24;288(21):14993-5005. doi: 10.1074/jbc.M112.446237. Epub 2013 Apr 9.
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RNA toxicity in polyglutamine disorders: concepts, models, and progress of research.多聚谷氨酰胺疾病中的 RNA 毒性：概念、模型和研究进展。
J Mol Med (Berl). 2013 Jun;91(6):683-91. doi: 10.1007/s00109-013-1016-2. Epub 2013 Mar 20.
5
Ganglioside GM1 induces phosphorylation of mutant huntingtin and restores normal motor behavior in Huntington disease mice.神经节苷脂 GM1 诱导突变 huntingtin 的磷酸化，恢复亨廷顿病小鼠的正常运动行为。
Proc Natl Acad Sci U S A. 2012 Feb 28;109(9):3528-33. doi: 10.1073/pnas.1114502109. Epub 2012 Feb 13.
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Polyglutamine neurodegeneration: expanded glutamines enhance native functions.多聚谷氨酰胺神经退行性变：扩展的谷氨酰胺增强了天然功能。
Curr Opin Genet Dev. 2012 Jun;22(3):251-5. doi: 10.1016/j.gde.2012.01.001. Epub 2012 Jan 25.
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Kinase inhibitors modulate huntingtin cell localization and toxicity.激酶抑制剂调节亨廷顿蛋白细胞定位和毒性。
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Mechanisms of trinucleotide repeat instability during human development.人类发育过程中三核苷酸重复不稳定的机制。
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Huntingtin has a membrane association signal that can modulate huntingtin aggregation, nuclear entry and toxicity.亨廷顿蛋白具有一个膜关联信号，该信号可调节亨廷顿蛋白的聚集、核内进入及毒性。
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