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
Autores principales: 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: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2017
Materias:
Plant Science
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5478728/
https://www.ncbi.nlm.nih.gov/pubmed/28680430
http://dx.doi.org/10.3389/fpls.2017.01026

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5478728/
https://www.ncbi.nlm.nih.gov/pubmed/28680430
http://dx.doi.org/10.3389/fpls.2017.01026

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