Sphingolipid activator proteins (SAPs) in neuronal ceroid lipofuscinoses (NCL).
Sphingolipid activator proteins (SAPs) in neuronal ceroid lipofuscinoses (NCL).
复制标题
神经元蜡质脂褐质 (NCL) 中的鞘脂激活蛋白 (SAP)。
DOI：
10.1055/s-2007-973667
复制
发表时间：
1997
期刊：
影响因子：
1.4
通讯作者：
Haltia,M
中科院分区：
文献类型：
--
作者：
Tyynela,J;Suopanki,J;Baumann,M;Haltia,M
根据储存物质的主要成分，神经元蜡样脂褐素(NCL)可分为两类：一类储存线粒体三磷酸腺苷合成酶c亚单位，另一类储存鞘磷脂激活蛋白(SAPS)。后者以人类婴儿NCL、先天性绵羊NCL和犬NCL为代表。少量的SAP也在大多数其他形式的NCL中积累。讨论了SAP及其功能、在不同形态的NCL中的存在，以及SAP与与INCL有关的棕榈酰基蛋白硫酯酶的关系。
Based on the predominant component of the storage material the neuronal ceroid lipofuscinoses (NCL) can be divided into two categories: one storing mitochondrial ATP synthase subunit c and the other storing sphingolipid activator proteins (SAPs). The latter group is represented by the human infantile NCL (INCL), a congenital ovine NCL, and a canine NCL. Small amounts of SAPs also accumulate in most other forms of NCL. The SAPs, their functions and occurrence in different forms of NCL, as well as the relationship between SAPs and palmitoyl protein thioesterase, an enzyme implicated in INCL, are discussed.