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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...
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. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2017
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Materias: | |
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 |
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