Several neurodegenerative diseases result from the aggregation of polyglutamine repeat proteins into insoluble neuronal intranuclear inclusions. The invention provides methods with which to study the processes of these diseases, including methods for solubilizing polypeptides containing a polyglutamine repeat sequence, for storing these polyglutamine polypeptides and inhibit their spontaneous aggregation, for making the aggregates of polyglutamine polypeptides, for assaying the extension of existing polyglutamine aggregates, for determining the ability of a chemical compound to inhibit aggregation, and for inhibiting aggregation of polyglutamine polypeptides. The invention further provides materials with which to study these diseases including a synthetic aggregate that have a capability to recruit additional monomeric polyglutamine polypeptides and chemical compounds that inhibit the formation and/or extension of polyglutamine aggregates.
一些神经退行性疾病是由多谷
氨酰胺重复蛋白聚集成不溶性神经元核内包涵体引起的。本发明提供了研究这些疾病过程的方法,包括溶解含有多谷
氨酰胺重复序列的
多肽的方法、储存这些多谷
氨酰胺
多肽并抑制其自发聚集的方法、制造多谷
氨酰胺
多肽聚集体的方法、检测现有多谷
氨酰胺聚集体扩展的方法、确定化合物抑制聚集能力的方法以及抑制多谷
氨酰胺
多肽聚集的方法。本发明进一步提供了用于研究这些疾病的材料,包括具有招募额外多聚谷
氨酰胺
多肽单体能力的合成聚集体和抑制多聚谷
氨酰胺聚集体形成和/或延伸的化合物。