CHROMATOGRAPHIC SEPARATION OF HUMAN BRAIN GANGLIOSIDES

CHROMATOGRAPHIC SEPARATION OF HUMAN BRAIN GANGLIOSIDES
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DOI:
10.1111/j.1471-4159.1963.tb08933.x
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发表时间:
1963-01-01
影响因子:
4.7
通讯作者:
SVENNERHOLM, L
SVENNERHOLM, L
中科院分区:
医学2区
文献类型:
--
作者:
SVENNERHOLM, L

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神经节苷脂已从胎儿、新生儿和成人正常人脑中分离出来。在最后一个例子中,神经节苷脂也是从分离的灰质和白质中获得的。用定量分析方法和薄层层析法对神经节苷脂的结构进行了研究。对间苯二酚测定唾液酸的方法进行了改进,并对神经节苷脂-唾液酸最常用的分析方法进行了比较。介绍了一种分离神经节苷脂的新方法。以纸卷柱为支撑介质,丙醇-水混合物为溶剂。这种类型的柱已被发现非常适合于将神经节苷脂分离成单唾液酸神经节苷脂和双唾液酸神经节苷脂部分,并用于后者的后续分级。通过唾液酸酶的酶解表明,90%的神经节苷脂来自相同的“母体”神经节苷脂-N-四糖-N-乙酰神经氨酸,在大多数神经节苷脂中,还有一或两个摩尔的N-乙酰神经氨酸;这些神经节苷脂最有可能在末端与半乳糖分子的C3和C6结合。这一假设得到了二和三唾液酸神经节苷脂的酸和酶水解的支持。在一例婴儿无黑神经症(Tay-Sachs)中,乙酰鞘氨醇-N-三糖-N-乙酰神经氨酸占神经节苷脂总量的90%以上。这种神经节苷脂只有3%-6%存在于正常人脑中。在1例幼年型无性痴呆(Spielmeyer-Vogt)中,神经节苷脂模式是正常的。
Gangliosides have been isolated from fetal, newborn and adult normal human brains. In the last instance gangliosides were also obtained from isolated grey and white matter. The ganglioside pattern has been studied with quantitative analytical methods and thin-layer chromatography. The resorcinol assay for sialic acids has been modified and the most common methods for the analysis of ganglioside-sialic acid have been compared. A new chromatographic method for separation of gangliosides is described. A paper roll column is used as a supporting medium and propanol-water mixtures as solvents. This type of column has been found very suitable for the separation of the gangliosides into a monosialo- and a disialoganglioside fraction and for the subsequent fractionation of the latter. By enzymic hydrolysis with sialidase it has been shown that 90 per cent of the gangliosides are derived from the same "parent ganglioside" acyl-sphingosine-N-tetrose-N-acetylneuraminic acid to which, inmost gangliosides, a further one or two moles of N-acetylneuraminic acid are bound; these are most likely bound to C3 and C6 of the galactose molecule in the terminal position. This assumption is supported by acid and enzymic hydrolysis of the di-and trisialogangliosides. In a case of infantile amaurotic idiocy (Tay-Sachs) acyl-sphingosine-N-triose-N-acetylneuraminic acid constituted more than 90 per cent of total gangliosides. Only 3-6 per cent of this ganglioside was found in normal human brains. In a case of juvenile amaurotic idiocy (Spielmeyer-Vogt) the ganglioside pattern was normal.