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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Vydáno v:Front Plant Sci
Hlavní autoři: 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.
Médium: Artigo
Jazyk:Inglês
Vydáno: Frontiers Media S.A. 2017
Témata:
Plant Science
On-line přístup: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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