Article
A Specific Activity-Based Probe to Monitor Family GH59 Galactosylceramidase, the Enzyme Deficient in Krabbe Disease.
Chembiochem : a European journal of chemical biology - 16 Feb 2017
Marques André R A, Willems Lianne I, Herrera Moro Daniela, Florea Bogdan I, Scheij Saskia, Ottenhoff Roelof, van Roomen Cindy P A A, Verhoek Marri, Nelson Jessica K, Kallemeijn Wouter W, Biela-Banas Anna, Martin Olivier R, Cachón-González M Begoña, Kim Nee Na, Cox Timothy M, Boot Rolf G, Overkleeft Herman S, Aerts Johannes M F G
Abstract excerpt
Galactosylceramidase (GALC) is the lysosomal β-galactosidase responsible for the hydrolysis of galactosylceramide. Inherited deficiency in GALC causes Krabbe disease, a devastating neurological disorder characterized by accumulation of galactosylceramide and its deacylated counterpart, the toxic sphingoid base galactosylsphingosine (psychosine). We report the design and application of a fluorescently tagged...
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