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Determination of globotriaosylceramide analogs in the organs of a mouse model of Fabry disease

Fabry disease is a heritable lipid disorder caused by the low activity of α-galactosidase A and characterized by the systemic accumulation of globotriaosylceramide (Gb3). Recent studies have reported a structural heterogeneity of Gb3 in Fabry disease, including Gb3 isoforms with different fatty acid...

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Detalles Bibliográficos
Autores principales: Ishii, Satoshi, Taguchi, Atsumi, Okino, Nozomu, Ito, Makoto, Maruyama, Hiroki
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Society for Biochemistry and Molecular Biology 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7186183/
https://www.ncbi.nlm.nih.gov/pubmed/32179651
http://dx.doi.org/10.1074/jbc.RA120.012665