Section 25
Chapter 24,328

Chromatographic Studies of Normal and Tay-Sachs Gangliosides (Peptide-Strandin)

Saifer, A.; Robin, M.; Volk, B.W.

Journal of Neurochemistry 10: 577-586


ISSN/ISBN: 0022-3042
PMID: 14062003
Accession: 024327174

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Gangliosides (peptide-stran-din) were isolated from grey matter of normal human and Tay-Sachs brain and purified. With thin-layer chromatography, normal human strandin (NHS) could be separated with various developing solvents, e.g. 70% n[long dash]propanol, into at least four components, whereas the Tay-Sachs strandin (TSS) yielded mainly (90%) a single component with a mobility greater than that of any of the normal fractions. Analysis of the chemical composition of purified NHS and TSS was undertaken in an attempt to determine the basis of the chromatographic differentiation. No appreciable differences were found in their fatty acid composition as determined by gas-liquid chromatography. Amino acid analysis of their peptide chains by means of automatic ion-exchange chromatography showed no qualitative differences in the twenty amino acids found, but considerably greater amounts of such compounds were present in NHS than in TSS. However, after hydrolysis, the latter contained appreciably greater amounts of carbohydrates, including glucose, galactose, galactosamine and N-acetylneuraminic acid. The relationships of these findings to those reported by other investigators and to the possible enzymic defect in Tay-Sachs disease are discussed.

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