Distributions of Globotriaosylceramide Isoforms, and Globotriaosylsphingosine and Its Analogues in an α-Galactosidase A Knockout Mouse, a Model of Fabry Disease

Fabry disease is caused by deficient activity of α-galactosidase A (GLA) and characterized by systemic accumulation of glycosphingolipids, substrates of the enzyme. To gain insight into the pathogenesis of Fabry disease based on accumulated substrates, we examined the tissue and plasma distributions...

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Detalles Bibliográficos
Autores principales: Sueoka, Hideaki, Aoki, Mikio, Tsukimura, Takahiro, Togawa, Tadayasu, Sakuraba, Hitoshi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2015
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4685999/
https://www.ncbi.nlm.nih.gov/pubmed/26661087
http://dx.doi.org/10.1371/journal.pone.0144958

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4685999/
https://www.ncbi.nlm.nih.gov/pubmed/26661087
http://dx.doi.org/10.1371/journal.pone.0144958

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