Castaño E M, Frangione B
Department of Pathology, New York Medical Center, New York, USA.
Curr Opin Neurol. 1995 Aug;8(4):279-85. doi: 10.1097/00019052-199508000-00006.
Amyloidosis and prionosis are disorders of protein conformation. The general mechanisms involved in amyloidogenesis are reviewed here. Recent progress in the molecular pathogenesis of cerebral amyloids is illustrated by three genetic disorders: hereditary amyloid angiopathies of Icelandic and Dutch origins and Gerstmann-Sträussler-Scheinker disease.
淀粉样变性和朊病毒病是蛋白质构象紊乱疾病。本文综述了淀粉样蛋白生成所涉及的一般机制。三种遗传性疾病说明了脑淀粉样蛋白分子发病机制的最新进展:源自冰岛和荷兰的遗传性淀粉样血管病以及格斯特曼-施特劳斯勒-申克病。