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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Detalles Bibliográficos
Publicado en: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: Frontiers Media S.A. 2017
Assuntos:
Plant Science
Acceso en liña: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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