Palo J, Elovaara I, Haltia M, Kin N Y, Wolfe L S
Neurology. 1982 Sep;32(9):1035-8. doi: 10.1212/wnl.32.9.1035.
We used ultracentrifugation to isolate autofluorescent storage particles from the brains of patients with infantile type of neuronal ceroid lipofuscinosis (Haltia-Santavuori). The particles differed from those of the late infantile (Jansky-Bielschowsky) and juvenile (Batten-Spielmeyer-Sjögren) types and retained the characteristic appearance--large, partly membrane-bound conglomerates of spherical globules with a uniform finely granular internal structure--despite the vigorous procedure. The sedimentation properties of the particles also differed from the two other forms, but dolichols are found in the particles of all three diseases.
我们采用超速离心法从患有婴儿型神经元蜡样脂褐质沉积症（哈尔蒂亚 - 桑塔沃里型）患者的大脑中分离出自发荧光储存颗粒。这些颗粒与晚发型婴儿型（扬斯基 - 比尔肖夫斯基型）和青少年型（巴顿 - 施皮尔曼 - 舍格伦型）的颗粒不同，尽管经过了剧烈的处理过程，但仍保留了其特征性外观——由球形小体组成的大的、部分有膜包裹的聚集体，内部结构均匀且呈细颗粒状。这些颗粒的沉降特性也与其他两种形式不同，但在所有这三种疾病的颗粒中都发现了多萜醇。
