Beta-glucosidase 1 (GBA1) is a second bile acid β-glucosidase in addition to β-glucosidase 2 (GBA2). Study in β-glucosidase deficient mice and humans

► Lysosomal glucosylceramidase (beta-glucosidase 1, GBA1) is in addition to the non-lysosomal glucosylceramidase (beta-glucosidase 2, GBA2) a second bile acid beta-glucosidase. ► Using bile acid β-glucosides as substrate, GBA1 and GBA2 can be determined in different tissues. ► GBA2 activity is in brain tissue higher as compared to GBA1 activity. ► Living primary fibroblasts metabolise exogenous bile acid β-glucosides.