Liberski P P
Department of Oncology, Medical Academy, Lódź, Poland.
Acta Neurobiol Exp (Wars). 1993;53(1):337-49.
I report here the basic concepts of the transmissible and non-transmissible cerebral amyloidoses. The pathogenesis of both types of brain amyloidoses consist of a synthesis of amyloid precursor followed by its processing to yield a final postranslationally modified deposit. PrPc and APP serve as precursor proteins while modified PrP (PrPsc and PrP 27-30) and beta-A4 as final deposits in transmissible and non-transmissible brain amyloidoses, respectively. The hallmark of both types of brain amyloidoses is amyloid deposits immunoreactive to appropriate amyloids. Other, mostly unspecific, neuropathological phenomena as neurofibrillary tangles, dystrophic neurites and granulovacuolar degeneration are also discussed. The only disease-specific structure for transmissible cerebral amyloidoses are tubulovesicular structures of unknown biological significance. They may even be the infectious virus.
我在此报告可传播性和非传播性脑淀粉样变性的基本概念。这两种类型脑淀粉样变性的发病机制均包括淀粉样前体蛋白的合成，随后对其进行加工以产生最终经翻译后修饰的沉积物。朊蛋白（PrPc）和淀粉样前体蛋白（APP）分别作为可传播性和非传播性脑淀粉样变性中的前体蛋白，而修饰后的朊蛋白（PrPsc和PrP 27-30）和β-淀粉样蛋白（beta-A4）分别作为最终沉积物。这两种类型脑淀粉样变性的标志都是对适当淀粉样蛋白具有免疫反应性的淀粉样沉积物。还讨论了其他大多是非特异性的神经病理现象，如神经原纤维缠结、营养不良性神经突和颗粒空泡变性。可传播性脑淀粉样变性唯一具有疾病特异性的结构是生物学意义不明的管泡状结构。它们甚至可能就是感染性病毒。
