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Hepatic galactosylceramide in globoid cell leukodystrophy (Krabbe's disease)
Author(s) -
Dawson Glyn
Publication year - 1973
Publication title -
lipids
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.601
H-Index - 120
eISSN - 1558-9307
pISSN - 0024-4201
DOI - 10.1007/bf02531813
Subject(s) - leukodystrophy , krabbe disease , clinical chemistry , lipidology , sphingolipid , cell , medicine , endocrinology , chemistry , biology , biochemistry , disease
Glycosphingolipids were isolated from the liver of four patients with globoid cell leukodystrophy and compared to those from normal liver. The ratio of glucosylceramide to galactosylceramide was 0.8, 1.2, 1.3 and 7.5 in the four cases studied, compared to a value of greater than 10 in liver from control patients of the same age and sex. In addition to this accumulation of galactosylceramide, all four livers showed an elevated level of sulfatide, and in one case trihexosylceramide and globoside were also markedly elevated.