Departamento de Química Física Aplicada, Universidad Autónoma de Madrid, Madrid, Spain.
Adv Protein Chem Struct Biol. 2013;91:37-63. doi: 10.1016/B978-0-12-411637-5.00002-0.
Aggregation, nuclear location, and nucleic acid interaction are common features shared by a number of proteins related to neurodegenerative diseases, including Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis, transmissible spongiform encephalopathy, Huntington's disease, spinobulbar muscular atrophy, dentatorubro-pallidoluysian atrophy, and several spinocerebellar ataxias. β-Amyloid peptides, tau protein, α-synuclein, superoxide dismutase1, prion protein, huntingtin, atrophin1, androgen receptor, and several ataxins are proteins prone to becoming aggregated, to translocate inside cell nucleus, and to bind DNA. In this chapter, we review those common features suggesting that neurological diseases too may share a transcriptional disorder, making it an important contribution to the origin of the disease.
聚集、核定位和核酸相互作用是与神经退行性疾病相关的许多蛋白质的共同特征，包括阿尔茨海默病、帕金森病、肌萎缩侧索硬化症、传染性海绵状脑病、亨廷顿病、橄榄脑桥小脑萎缩、齿状核红核苍白球路易体萎缩和几种脊髓小脑共济失调。β-淀粉样肽、tau 蛋白、α-突触核蛋白、超氧化物歧化酶 1、朊病毒蛋白、亨廷顿蛋白、atrophin1、雄激素受体和几种ataxin 是易于聚集、易转位到细胞核内并与 DNA 结合的蛋白质。在本章中，我们回顾了这些共同特征，表明神经退行性疾病也可能存在转录失调，这是疾病起源的重要贡献。
