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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Detalhes bibliográficos

Principais autores:	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
Formato:	Artigo
Idioma:	Inglês
Publicado em:	Frontiers Media S.A. 2017-06-01
coleção:	Frontiers in Plant Science
Assuntos:	α-galactosidase α-N-acetyl-galactosaminidase Fabry disease therapy recombinant enzyme Nicotiana benthamiana
Acesso em linha:	http://journal.frontiersin.org/article/10.3389/fpls.2017.01026/full
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