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Article

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

Journal of lipid research - 1 Dec 2001

Whitfield P D, Sharp P C, Taylor R, Meikle P

Abstract excerpt

Globoid cell leukodystrophy (Krabbe disease) is an autosomal recessive inherited neurodegenerative disorder caused by the deficiency of the lysosomal enzyme beta-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...

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PMID: 11734583

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

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