Glycosphingolipid storage in Fabry mice extends beyond globotriaosylceramide and is affected by ABCB1 depletion

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 gly...

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Detalhes bibliográficos
Publicado no:Future Sci OA
Main Authors: Kamani, Mustafa A, Provençal, Philippe, Boutin, Michel, Pacienza, Natalia, Fan, Xin, Novak, Anton, Huang, Tonny C, Binnington, Beth, Au, Bryan C, Auray-Blais, Christiane, Lingwood, Clifford A, Medin, Jeffrey A
Formato: Artigo
Idioma:Inglês
Publicado em: Future Science Ltd 2016
Assuntos:
Research Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC5242178/
https://ncbi.nlm.nih.gov/pubmed/28116130
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.4155/fsoa-2016-0027
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