Zoghbi H Y, Orr H T
Howard Hughes Medical Institute Baylor College of Medicine Houston, Texas 77030, USA.
Curr Opin Neurobiol. 1999 Oct;9(5):566-70. doi: 10.1016/S0959-4388(99)00013-6.
Neuronal aggregates of the disease-causing protein, often in the nucleus of affected cells, are a pathological hallmark of the neurodegenerative diseases known as polyglutamine disorders. It was suggested that these nuclear aggregates are the cause of these disorders. However, recent evidence suggests that the aggregates, in fact, are not the pathogenic basis and, instead, may play a role in sequestration of the pathogenic protein.
致病蛋白的神经元聚集体，通常存在于受影响细胞的细胞核中，是被称为多聚谷氨酰胺疾病的神经退行性疾病的病理标志。有人认为这些核聚集体是这些疾病的病因。然而，最近的证据表明，这些聚集体实际上并不是致病基础，相反，它们可能在隔离致病蛋白方面发挥作用。
