Neuropharmacology & Neurotherapeutics
Mar. 02, 2021
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The enzyme alpha-galactosidase A cleaves off the third sugar residue. In Fabry disease this enzyme is deficient, and therefore the substrate Gb3 accumulates in the lysosomes of almost all cell types. (Source: Amodio F, Caiazza M, Monda E, et al. An overview of molecular mechanisms in Fabry disease. Biomolecules 2022;12:1460. Creative Commons Attribution [CC BY] license, creativecommons.org/licenses/by/4.0.)