PMID: 2120388Nov 1, 1990Paper

Saposins (sphingolipid activator proteins) in the twitcher mutant mouse

Journal of Neurochemistry
H ShigematsuJ S O'Brien

Abstract

The twitcher mutant mouse, the animal model of Krabbe disease (human globoid cell leukodystrophy), is characterized by apparent deficiency of galactosylceramide beta-galactosidase activity. Saposin A and C, the heat-stable small sphingolipid activator glycoproteins, stimulate the activity of galactosylceramide beta-galactosidase as well as glucosylceramide beta-glucoside. The role of these saposins in the twitcher mutation was investigated. Boiled supernatant fractions, which contained saposins, were prepared from homogenates of twitcher brain, liver, kidney, and spleen. These preparations showed an almost identical effect on the activity of purified glucosylceramide beta-glucosidase (measured by hydrolysis of 4-methylumbelliferyl-beta-glucoside) with similar preparations from control tissues. The effect on the activity of galactosylceramide beta-galactosidase as well as 4-methylumbelliferyl-beta-glucoside beta-glucosidase in the twitcher brain and liver homogenates by authentic saposin A and C was similar to that in control tissues. These results suggest that the twitcher mutation does not affect the concentrations of saposin A or C or their interaction with galactosylceramide beta-galactosidase.

References

May 1, 1990·Proceedings of the National Academy of Sciences of the United States of America·S MorimotoY Kishimoto
May 1, 1989·Proceedings of the National Academy of Sciences of the United States of America·S MorimotoY Kishimoto
Apr 15, 1981·Archives of Biochemistry and Biophysics·B L Berent, N S Radin
Sep 14, 1982·Biochimica Et Biophysica Acta·D A WengerS Roth

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Citations

Sep 12, 2006·FEBS Letters·Walter M HolleranYoshikazu Uchida
Sep 18, 2016·Journal of Neuroscience Research·Keri R Maher, Andrew M Yeager

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