Vishnevskaia A B, Kushnirov V V, Ter-Avanesian M D
Mol Biol (Mosk). 2007 Mar-Apr;41(2):346-54.
More than 20 human diseases are related to protein misfolding which causes formation of amyloids, fibrillar aggregates of normally soluble proteins. Such diseases are called amyloid diseases or amyloidoses. Of them only prion diseases are transmissible. Amyloids of the prion type are described in lower eukaryotes. However, in contrast to mammalian prions, which cause incurable neurodegenerative diseases, prions of lower eukaryotes are related to some non-chromosomally inherited phenotypic traits. Here we summarize the results of studies of prions of the yeast Saccharomyces cerevisiae and of the use of yeast model for investigation of some human amyloidoses, such as prion diseases, Alzheimer's, Parkinson's, and Huntington's diseases.
20多种人类疾病与蛋白质错误折叠有关，蛋白质错误折叠会导致淀粉样蛋白的形成，淀粉样蛋白是正常可溶性蛋白质的纤维状聚集体。这类疾病被称为淀粉样疾病或淀粉样变性。其中只有朊病毒疾病具有传染性。在低等真核生物中描述了朊病毒类型的淀粉样蛋白。然而，与导致无法治愈的神经退行性疾病的哺乳动物朊病毒不同，低等真核生物的朊病毒与一些非染色体遗传的表型特征有关。在这里，我们总结了对酿酒酵母朊病毒的研究结果，以及利用酵母模型研究某些人类淀粉样变性疾病的情况，如朊病毒疾病、阿尔茨海默病、帕金森病和亨廷顿舞蹈症。
