Increased globotriaosylceramide levels in a transgenic mouse expressing human α1,4-galactosyltransferase and a mouse model for treating Fabry disease

Fabry disease is a lysosomal storage disorder caused by an α-galactosidase A (α-Gal A) deficiency and resulting in the accumulation of glycosphingolipids, predominantly globotriaosylceramide (Gb3). A transgenic mouse expressing the human α-Gal A R301Q mutant in an α-Gal A-knockout background (TgM/KO...

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Détails bibliographiques
Auteurs principaux: Shiozuka, Chikara, Taguchi, Atsumi, Matsuda, Junichiro, Noguchi, Yoko, Kunieda, Takanori, Uchio-Yamada, Kozue, Yoshioka, Hidekatsu, Hamanaka, Ryoji, Yano, Shinji, Yokoyama, Shigeo, Mannen, Kazuaki, Kulkarni, Ashok B., Furukawa, Koichi, Ishii, Satoshi
Format: Artigo
Langue:Inglês
Publié: Oxford University Press 2011
Sujets:
Regular Papers
Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC3031308/
https://ncbi.nlm.nih.gov/pubmed/20961863
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/jb/mvq125
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