Haan J, Hardy J A, Roos R A
Dept of Neurology, University Hospital, Leiden, The Netherlands.
Trends Neurosci. 1991 Jun;14(6):231-4. doi: 10.1016/0166-2236(91)90120-j.
Alzheimer's disease is now commonly regarded as a form of 'amyloid encephalopathy'. Amyloid deposits in the cerebral blood vessels and parenchyma consist mainly of a unique protein called amyloid beta protein (A beta P), which has a molecular weight of 4 kDa and is 42 amino acids long. These deposits are thought to be of pathogenetic importance in Alzheimer's disease. Recently, therefore, attention has been focused on the process of turnover of the precursor of A beta P to amyloid fibrils, and the deposition and persistence of A beta P in this disease. The study of several other diseases with cerebral A beta P deposition can be informative in this respect, because they allow the comparison of different pathogenetic mechanisms that lead to this type of deposition. One of these diseases is hereditary cerebral hemorrhage with amyloidosis- Dutch type (HCHWA-D), which is the subject of this review.
阿尔茨海默病现在通常被视为一种“淀粉样蛋白脑病”。淀粉样蛋白在脑血管和脑实质中的沉积主要由一种名为β淀粉样蛋白（AβP）的独特蛋白质组成，其分子量为4 kDa，由42个氨基酸组成。这些沉积物被认为在阿尔茨海默病的发病机制中具有重要意义。因此，最近人们的注意力集中在AβP前体向淀粉样纤维的周转过程，以及AβP在这种疾病中的沉积和持续存在。对其他几种伴有脑AβP沉积的疾病的研究在这方面可能会提供信息，因为它们可以比较导致这种类型沉积的不同发病机制。其中一种疾病是荷兰型遗传性脑出血伴淀粉样变性（HCHWA-D），这也是本综述的主题。
