Chapter Two Chapter Two Identification and use of biomarkers in Gaucher disease and other lysosomal storage diseases
J. Aerts, C. Hollak, M. V. van Breemen et al.
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Chapter Thirteen Chapter Thirteen
J. M. Aerts, M. V. van Breemen, Anton P. Bussink et al.
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Open access
Aug 2026
Unraveling A4GALT Mechanism and Its Modulation With Adamantyl-Galactosylceramide Analogues: Advancing Fabry Disease Therapeutic Strategies.
The molecular mechanism of human A4GALT is revealed at atomic detail using QM/MM simulations, revealing a conformational rearrangement involving a 310-helix that stabilizes the donor substrate and promotes a front-face SNi-like catalytic mechanism, in which a short-lived oxocarbenium-ion intermediate forms.
Nicky de Koster, Òscar Vidal-Gironès, Rowan de Graaf et al.
· Angewandte Chemie · 0 citations