赫尔曼斯基-普德拉克综合征及神经元蜡样脂褐质沉积症中的蜡样脂褐质
Ceroid lipofuscin in Hermansky-Pudlak syndrome and neuronal ceroid lipofuscinoses.
作者信息
Puranen M
机构信息
University of Kuopio, Faculty of Dentistry, Finland.
出版信息
Proc Finn Dent Soc. 1990;86(2):99-102.
PMID:2287615
Abstract
Ceroid lipofuscin has been found to accumulate in body tissues in two types of storage disease, the Hermansky-Pudlak syndrome (HPS) and neuronal ceroid lipofuscinoses (NCL), which exhibit different clinical patterns and also differ as regards the site of pigment accumulation. The storage material in both diseases is similar physically and biochemically.
摘要
已发现脂褐素在两种贮积病的身体组织中蓄积，这两种疾病分别是赫尔曼斯基-普德拉克综合征（HPS）和神经元蜡样脂褐质沉积症（NCL），它们表现出不同的临床模式，并且在色素蓄积部位也存在差异。这两种疾病中的贮积物质在物理和生化性质上相似。
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