Human Alpha Galactosidases Transiently Produced in Nicotiana benthamiana Leaves: New Insights in Substrate Specificities with Relevance for Fabry Disease

Deficiency of α-galactosidase A (α-GAL) causes Fabry disease (FD), an X-linked storage disease of the glycosphingolipid globtriaosylcerammide (Gb3) in lysosomes of various cells and elevated plasma globotriaosylsphingosine (Lyso-Gb3) toxic for podocytes and nociceptive neurons. Enzyme replacement th...

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Detalhes bibliográficos
Publicado no:Front Plant Sci
Main Authors: Kytidou, Kassiani, Beenakker, Thomas J. M., Westerhof, Lotte B., Hokke, Cornelis H., Moolenaar, Geri F., Goosen, Nora, Mirzaian, Mina, Ferraz, Maria J., de Geus, Mark, Kallemeijn, Wouter W., Overkleeft, Herman S., Boot, Rolf G., Schots, Arjen, Bosch, Dirk, Aerts, Johannes M. F. G.
Formato: Artigo
Idioma:Inglês
Publicado em: Frontiers Media S.A. 2017
Assuntos:
Plant Science
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC5478728/
https://ncbi.nlm.nih.gov/pubmed/28680430
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3389/fpls.2017.01026
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