Prelli F, Castaño E M, van Duinen S G, Bots G T, Luyendijk W, Frangione B
Department of Pathology, New York University Medical Center, NY 10016.
Biochem Biophys Res Commun. 1988 Mar 30;151(3):1150-5. doi: 10.1016/s0006-291x(88)80486-8.
Hereditary cerebral hemorrhage with amyloidosis in patients of Dutch origin is an autosomal-dominant type of amyloidosis restricted to the small vasculature of the brain and clinically characterized by recurrent strokes. Amyloid fibrils from the leptomeninges of two patients were isolated and the primary structure determined. The complete sequence of the amyloid protein shows homology to the vascular (beta-protein) and plaque amyloid (A4-protein) obtained from Alzheimer's Disease. However, it is three residues shorter (39 instead of 42) than that reported for the plaque amyloid. The difference at the carboxy terminal may reflect specific degradation that occurs in the vessel wall and not in the brain parenchyma.
源于荷兰患者的遗传性脑出血伴淀粉样变性是一种常染色体显性淀粉样变性，仅限于脑小血管，临床特征为复发性中风。从两名患者的软脑膜中分离出淀粉样原纤维并确定其一级结构。淀粉样蛋白的完整序列与从阿尔茨海默病中获得的血管（β蛋白）和斑块淀粉样蛋白（A4蛋白）具有同源性。然而，它比报道的斑块淀粉样蛋白短三个残基（39个而不是42个）。羧基末端的差异可能反映了在血管壁而非脑实质中发生的特定降解。
