Saposins (sphingolipid activator proteins) in the twitcher mutant mouse

H Shigematsu, S Morimoto, Y Kishimoto, S Weiler, J Tomich, J Barranger, M Shinohara, A M Yeager, J S O'Brien

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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.

Original languageEnglish
Pages (from-to)1659-62
Number of pages4
JournalJournal of Neurochemistry
Volume55
Issue number5
Publication statusPublished - Nov 1990

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Sphingolipid Activator Proteins
Saposins
Galactosylceramidase
Globoid Cell Leukodystrophy
Glucosides
Liver
Brain
Glucosylceramidase
Tissue
Glucosylceramides
Mutation
Sphingolipids
beta-Glucosidase
Hydrolysis
Glycoproteins
Animals
Spleen
Animal Models
Hot Temperature

Cite this

Shigematsu, H., Morimoto, S., Kishimoto, Y., Weiler, S., Tomich, J., Barranger, J., ... O'Brien, J. S. (1990). Saposins (sphingolipid activator proteins) in the twitcher mutant mouse. Journal of Neurochemistry, 55(5), 1659-62.

Saposins (sphingolipid activator proteins) in the twitcher mutant mouse. / Shigematsu, H; Morimoto, S; Kishimoto, Y; Weiler, S; Tomich, J; Barranger, J; Shinohara, M; Yeager, A M; O'Brien, J S.

In: Journal of Neurochemistry, Vol. 55, No. 5, 11.1990, p. 1659-62.

Research output: Contribution to journalArticle

Shigematsu, H, Morimoto, S, Kishimoto, Y, Weiler, S, Tomich, J, Barranger, J, Shinohara, M, Yeager, AM & O'Brien, JS 1990, 'Saposins (sphingolipid activator proteins) in the twitcher mutant mouse', Journal of Neurochemistry, vol. 55, no. 5, pp. 1659-62.
Shigematsu H, Morimoto S, Kishimoto Y, Weiler S, Tomich J, Barranger J et al. Saposins (sphingolipid activator proteins) in the twitcher mutant mouse. Journal of Neurochemistry. 1990 Nov;55(5):1659-62.
Shigematsu, H ; Morimoto, S ; Kishimoto, Y ; Weiler, S ; Tomich, J ; Barranger, J ; Shinohara, M ; Yeager, A M ; O'Brien, J S. / Saposins (sphingolipid activator proteins) in the twitcher mutant mouse. In: Journal of Neurochemistry. 1990 ; Vol. 55, No. 5. pp. 1659-62.
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N2 - 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.

AB - 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.

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