α- d -Gal-cyclophellitol cyclosulfamidate is a Michaelis complex analog that stabilizes therapeutic lysosomal α-galactosidase A in Fabry disease

Fabry disease is an inherited lysosomal storage disorder that is characterized by a deficiency in lysosomal α- d -galactosidase activity. One current therapeutic strategy involves enzyme replacement therapy, in which patients are treated with a recombinant enzyme. Co-treatment with enzyme active-sit...

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Veröffentlicht in:	Chemical science (Cambridge) 2019, Vol.10 (40), p.9233-9243
Hauptverfasser:	Artola, Marta, Hedberg, Christinne, Rowland, Rhianna J., Raich, Lluís, Kytidou, Kassiani, Wu, Liang, Schaaf, Amanda, Ferraz, Maria Joao, van der Marel, Gijsbert A., Codée, Jeroen D. C., Rovira, Carme, Aerts, Johannes M. F. G., Davies, Gideon J., Overkleeft, Herman S.
Format:	Artikel
Sprache:	eng
Schlagworte:	Chemical synthesis Crystallography Enzymes Galactose Galactosidase Mimicry NMR Nuclear magnetic resonance Organic chemistry
Online-Zugang:	Volltext
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