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

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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Bibliographic Details
Main Authors: Kassiani Kytidou, Thomas J. M. Beenakker, Lotte B. Westerhof, Cornelis H. Hokke, Geri F. Moolenaar, Nora Goosen, Mina Mirzaian, Maria J. Ferraz, Mark de Geus, Wouter W. Kallemeijn, Herman S. Overkleeft, Rolf G. Boot, Arjen Schots, Dirk Bosch, Johannes M. F. G. Aerts
Format: Article
Language:English
Published: Frontiers Media S.A. 2017-06-01
Series:Frontiers in Plant Science
Subjects:
α-galactosidase
α-N-acetyl-galactosaminidase
Fabry disease
therapy
recombinant enzyme
Nicotiana benthamiana
Online Access:http://journal.frontiersin.org/article/10.3389/fpls.2017.01026/full

Internet

http://journal.frontiersin.org/article/10.3389/fpls.2017.01026/full

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