Accumulation of dolichol-linked oligosaccharides in ceroid-lipofuscinosis (Batten disease).
Accumulation of dolichol-linked oligosaccharides in ceroid-lipofuscinosis (Batten disease).
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蜡质脂褐素沉着症（巴顿病）中多甘醇连接的寡糖的积累。
DOI：
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发表时间：
1988
期刊：
影响因子：
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通讯作者：
R. Pullarkat
中科院分区：
文献类型：
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作者：
N. Hall;A. D. Patrick;J. M. Opitz;J. Reynolds;R. Pullarkat
从蜡样脂褐素沉着症(CL)的病例中记录了一些组织中的磷酸化多醇化合物的积累，并对其复杂的碳水化合物结构进行了分析。从脑部部分纯化的二羟基焦磷化合物中释放低聚糖，可以通过温和的酸解或内切糖苷酶消化来进行。所释放的低聚糖的摩尔量与被分析的每个大脑中的P-DOLICHOL水平相对应。定性分析表明，脑寡糖由许多不同的组分组成，大小从4到14个单糖单元不等，还原末端含有甲壳二糖，含有7到8个单糖的物种可以被α-甘露糖苷酶完全消化为三糖。在CL组织中积累的化合物可能代表了一些已知参与蛋白质糖基化的脂质连接中间体，以及来自这些中间体的代谢物。结果表明，CL可能是由于代谢焦磷低聚糖的能力受损所致。
The accumulation of phosphorylated dolichol compounds in a number of tissues from cases of ceroid-lipofuscinosis (CL) is documented, together with an analysis of their complex carbohydrate structures. Oligosaccharides were released from dolichyl pyrophosphoryl compounds, partially purified from brain, either by mild acid hydrolysis or endoglucosaminidase digestion. The molar amounts of oligosaccharides released corresponded to the levels of P-dolichol in each brain analysed. Qualitative analysis indicated that the oligosaccharides from brain consist of a number of different components, ranging in size from four to fourteen monosaccharide units and containing chitobiose at the reducing terminal, and that the species containing seven or eight monosaccharides can be fully digested to a trisaccharide by alpha-mannosidase. The compounds that accumulate in CL tissues probably represent some of the lipid-linked intermediates known to be involved in the glycosylation of proteins, together with metabolites derived from these intermediates. The results suggest that CL might result from an impairment of the ability to metabolize dolichyl pyrophosphoryl oligosaccharides.
内切-β-N-乙酰氨基葡萄糖苷酶 H 裂解多甘基焦磷酸寡糖：糖释放的酶解和酸水解技术的比较。
影响因子：
3.9
作者：
Chalifour,RJ;Spiro,RG
通讯作者：
Spiro,RG