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Characterization of Neutral and Acidic Glycosphingolipids in Brains of Two Patients with GM 1 Gangliosidosis Type 1 and Type 2
Author(s) -
Ishikawa Yukitoshi,
Makita Akira,
Minami Ryoji
Publication year - 1985
Publication title -
journal of neurochemistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.75
H-Index - 229
eISSN - 1471-4159
pISSN - 0022-3042
DOI - 10.1111/j.1471-4159.1985.tb08730.x
Subject(s) - ganglioside , glycolipid , gangliosidosis , biochemistry , biology , stearic acid , tay sachs disease , endocrinology , medicine , chemistry , enzyme , disease , organic chemistry
Brains of two patients with GM 1 gangliosidosis type 1 and type 2, together with the age‐matched control brains, were analyzed for glycosphingolipids. Six species of neutral glycolipids, eight species of gangliosides, and sulfatide were isolated from the diseased brains and identified. In addition to GM 1 ganglioside and its asialo derivative, the diseased brains accumulated considerable amounts of gangliotriaosylceramide and glycolipids belonging to the globe series, the accumulation of which cannot be explained by deficient β‐galactosidase activity in this disease. GM 4 ganglioside was detected in the type 2 brain, but not in type 1. As to fatty acid composition of monohexosylceramides and sulfatide in the two diseased brains, stearic acid was more predominant in the type 1 brain than in the type 2 brain. In light of our previous observations on a Tay‐Sachs brain and present results, it appears that metabolism of the globo series glycolipids, which is active in normal brain at early infancy but inactive thereafter, remains in brains with GM 1 gangliosidosis (types 1 and 2) and Tay‐Sachs disease, reflecting a disturbance in development of the brain.