J. Lipid Res.
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Journal of Lipid Research, Vol. 42, 2092-2095, December 2001
Copyright © 2001 by Lipid Research, Inc.

Methods

Quantification of galactosylsphingosine in the twitcher mouse using electrospray ionization-tandem mass spectrometry

Phillip D. Whitfielda, Peter C. Sharpb, Rosanne Taylorc, and Peter Meiklea
a Lysosomal Diseases Research Unit, Department of Chemical Pathology, Women's and Children's Hospital, Adelaide, 72, King William Road, North Adelaide, South Australia 5006, Australia
b National Referral Laboratory, Department of Chemical Pathology, Women's and Children's Hospital, Adelaide, 72, King William Road, North Adelaide, South Australia 5006, Australia
c Faculty of Veterinary Science, University of Sydney, New South Wales, Australia

Correspondence to: Phillip D. Whitfield, To whom correspondence should be addressed., phillip.whitfield{at}adelaide.edu.au (E-mail)

Globoid cell leukodystrophy (Krabbe disease) is an autosomal recessive inherited neurodegenerative disorder caused by the deficiency of the lysosomal enzyme ß-galactosylceramidase. The pathogenesis of the disorder has been proposed to arise from the accumulation of the cytotoxic metabolite galactosylsphingosine (psychosine). The twitcher mouse is a naturally occurring murine model of globoid cell leukodystrophy. We have developed a rapid, sensitive, and specific mass spectrometric method for determining the galactosylsphingosine concentration in the tissues of twitcher mice. Galactosylsphingosine is extracted from the tissues in methanol, isolated using strong cation-exchange and C18 solid-phase extraction chromatography, and then directly analyzed using electrospray ionization-tandem mass spectrometry. A lactosylsphingosine internal standard has been employed for quantification.

The assay demonstrated significant accumulation of galactosylsphingosine in the brain, spinal cord, and kidney of twitcher mice. It is anticipated that this method may be of use in the monitoring of experimental therapies for globoid cell leukodystrophy. — Whitfield, P. D., P. C. Sharp, R. Taylor, and P. Meikle. Quantification of galactosylsphingosine in the twitcher mouse using electrospray ionization-tandem mass spectrometry. J. Lipid Res. 2001. 42: 2092–2095.

Supplementary key words: globoid cell leukodystrophy, Krabbe disease, lysoglycosphingolipids, psychosine, solid-phase extraction


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