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THE GAUCHER MOUSE: ADDITIONAL BIOCHEMICAL ALTERATIONS
Author(s) -
Stephens M. C.,
Bernatsky A.,
Legler G.,
Kanfer J. N.
Publication year - 1979
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.1471-4159.1979.tb04582.x
Subject(s) - lactosylceramide , medicine , spleen , endocrinology , gaucher's disease , ceramide , globotriaosylceramide , ganglioside , sphingolipid , acid phosphatase , chemistry , glycosphingolipid , biology , enzyme , biochemistry , enzyme replacement therapy , disease , apoptosis
— Additional biochemical alterations were observed in the Gaucher mouse. The activity of β‐xylosidase in brain, spleen and liver was considerably decreased in this animal model and acid phosphatase in plasma was increased about 4‐fold. The levels of the splenic hexosylceramides, glucosyl‐ceramide, lactosylceramide, trihexosylceramide, globoside and hematoside were all increased in comparison with controls. Brain ganglioside content was examined and very few differences were noted; however, there was a tendency towards slight elevations for the monosialogangliosides and GD 1a and lower values for GD 1b and GT.