α-Galactosidase A-deficient rats accumulate glycosphingolipids and develop cardiorenal phenotypes of Fabry disease

Fabry disease is an X-linked lysosomal storage disease caused by α-galactosidase A (α-Gal A) deficiency. Kidney and heart failure are frequent complications in adulthood and greatly contribute to patient morbidity and mortality. Because α-Gal A-deficient mouse models do not recapitulate cardiorenal...

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Detalhes bibliográficos
Publicado no:FASEB J
Main Authors: Miller, James J., Aoki, Kazuhiro, Mascari, Carly A., Beltrame, Angela K., Sokumbi, Olayemi, North, Paula E., Tiemeyer, Michael, Kriegel, Alison J., Dahms, Nancy M.
Formato: Artigo
Idioma:Inglês
Publicado em: Federation of American Societies for Experimental Biology 2019
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Research
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC6629127/
https://ncbi.nlm.nih.gov/pubmed/29979634
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1096/fj.201800771R
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