Glycosphingolipid Storage in Fabry Mice Extends Beyond Globotriaosylceramide and is Affected by ABCB1 Depletion | Zendy

Mustafa A. Kamani | Zendy; Philippe Provençal | Zendy; Michel Boutin | Zendy; Natalia Pacienza | Zendy; Xin Fan | Zendy; Anton Novak | Zendy; Tonny Chao Huang | Zendy; Beth Binnington | Zendy; B C Y Au | Zendy; Christiane AurayBlais | Zendy; Clifford A. Lingwood | Zendy; Jeffrey A. Medin | Zendy

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Glycosphingolipid Storage in Fabry Mice Extends Beyond Globotriaosylceramide and is Affected by ABCB1 Depletion

Author(s) -

Mustafa A. Kamani,

Philippe Provençal,

Michel Boutin,

Natalia Pacienza,

Xin Fan,

Anton Novak,

Tonny Chao Huang,

Beth Binnington,

B C Y Au,

Christiane AurayBlais,

Clifford A. Lingwood,

Jeffrey A. Medin

Publication year - 2016

Publication title -

future science oa

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 0.825

H-Index - 23

ISSN - 2056-5623

DOI - 10.4155/fsoa-2016-0027

Subject(s) - globotriaosylceramide , glycosphingolipid , fabry disease , sphingolipid , ceramide , fabry's disease , chemistry , biology , biochemistry , pathology , disease , medicine , apoptosis

Aim: Fabry disease is caused by α-galactosidase A deficiency leading to accumulation of globotriaosylceramide (Gb 3 ) in tissues. Clinical manifestations do not appear to correlate with total Gb 3 levels. Studies examining tissue distribution of specific acyl chain species of Gb 3 and upstream glycosphingolipids are lacking. Material & methods/Results: Thorough characterization of the Fabry mouse sphingolipid profile by LC-MS revealed unique Gb 3 acyl chain storage profiles. Storage extended beyond Gb 3 ; all Fabry tissues also accumulated monohexosylceramides. Depletion of ABCB1 had a complex effect on glycosphingolipid storage. Conclusion: These data provide insights into how specific sphingolipid species correlate with one another and how these correlations change in the α-galactosidase A-deficient state, potentially leading to the identification of more specific biomarkers of Fabry disease.

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