Haan J, Maat-Schieman M L, van Duinen S G, Jensson O, Thorsteinsson L, Roos R A
Department of Neurology, University Hospital Leiden, The Netherlands.
Acta Neurol Scand. 1994 May;89(5):367-71. doi: 10.1111/j.1600-0404.1994.tb02648.x.
Based on the recent discovery of co-localization of beta/A4 and cystatin C in cortical blood vessels of patients with cerebral hemorrhages due to sporadic amyloid angiopathy and patients with Alzheimer's disease we investigated the presence of these two proteins in the cortical blood vessels of patients suffering from hereditary cerebral hemorrhage with amyloidosis of the Dutch (n = 11) and the Icelandic (n = 2) type. The brains of three patients with sporadic cerebral amyloid angiopathy were also investigated. Blood vessels of the Dutch patients clearly showed immunostaining with beta/A4 as well as with cystatin C antibodies, whereas the blood vessels of Icelandic patients showed only staining with cystatin C. In one of the three sporadic amyloid angiopathy patients co-localization was shown as well. The co-localization of mutated beta/A4 with normal cystatin C in the Dutch patients suggests that cystatin C deposition occurs secondarily to beta/A4 deposition. This is probably also the case in sporadic amyloid angiopathy and Alzheimer's disease. Cystatin C deposition may play a role in the development of cerebral hemorrhages and leukoencephalopathy.
基于近期在散发性淀粉样脑血管病脑出血患者和阿尔茨海默病患者的皮质血管中发现β/A4和胱抑素C共定位，我们研究了荷兰型（n = 11）和冰岛型（n = 2）遗传性脑出血伴淀粉样变性患者的皮质血管中这两种蛋白的存在情况。还研究了3例散发性脑淀粉样血管病患者的大脑。荷兰患者的血管用β/A4抗体和胱抑素C抗体均显示出免疫染色，而冰岛患者的血管仅用胱抑素C染色显示阳性。在3例散发性淀粉样血管病患者中的1例也显示出共定位。荷兰患者中突变的β/A4与正常胱抑素C的共定位表明，胱抑素C沉积继发于β/A4沉积。散发性淀粉样血管病和阿尔茨海默病可能也是这种情况。胱抑素C沉积可能在脑出血和白质脑病的发生中起作用。
