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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Auteurs principaux:	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:	Artigo
Langue:	Inglês
Publié:	Frontiers Media S.A. 2017-06-01
Collection:	Frontiers in Plant Science
Sujets:	α-galactosidase α-N-acetyl-galactosaminidase Fabry disease therapy recombinant enzyme Nicotiana benthamiana
Accès en ligne:	http://journal.frontiersin.org/article/10.3389/fpls.2017.01026/full
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