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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Detalles Bibliográficos
Autores principales: 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: Online Artículo Texto
Lenguaje:English
Publicado: Future Science Ltd 2016
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5242178/
https://www.ncbi.nlm.nih.gov/pubmed/28116130
http://dx.doi.org/10.4155/fsoa-2016-0027

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5242178/
https://www.ncbi.nlm.nih.gov/pubmed/28116130
http://dx.doi.org/10.4155/fsoa-2016-0027

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