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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Bibliografske podrobnosti
izdano v: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.
Format: Artigo
Jezik:Inglês
Izdano: Federation of American Societies for Experimental Biology 2019
Teme:
Research
Online dostop: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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