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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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Wedi'i Gadw mewn:
Manylion Llyfryddiaeth
Cyhoeddwyd yn:PLoS One
Prif Awduron: Sueoka, Hideaki, Aoki, Mikio, Tsukimura, Takahiro, Togawa, Tadayasu, Sakuraba, Hitoshi
Fformat: Artigo
Iaith:Inglês
Cyhoeddwyd: Public Library of Science 2015
Pynciau:
Mynediad Ar-lein:https://ncbi.nlm.nih.gov/pmc/articles/PMC4685999/
https://ncbi.nlm.nih.gov/pubmed/26661087
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0144958
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