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Assessing the role of glycosphingolipids in the phenotype severity of Fabry disease mouse model

Fabry disease is caused by deficient activity of α-galactosidase A, an enzyme that hydrolyzes the terminal α-galactosyl moieties from glycolipids and glycoproteins, and subsequent accumulation of glycosphingolipids, mainly globotriaosylceramide (Gb(3)), globotriaosylsphingosine (lyso-Gb(3)), and gal...

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Detalhes bibliográficos
Publicado no:J Lipid Res
Main Authors: Jabbarzadeh-Tabrizi, Siamak, Boutin, Michel, Day, Taniqua S., Taroua, Mouna, Schiffmann, Raphael, Auray-Blais, Christiane, Shen, Jin-Song
Formato: Artigo
Idioma:Inglês
Publicado em: The American Society for Biochemistry and Molecular Biology 2020
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC7604726/
https://ncbi.nlm.nih.gov/pubmed/32868283
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1194/jlr.RA120000909
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