Lysosomal acid glucosylceramidase (GBA) is a ~59 kDa protein. Glucosylceramidase that catalyzes, within the lysosomal compartment, the hydrolysis of glucosylceramides/GlcCers (such as beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine) into free ceramides (such as N-acylsphing-4-enine) and glucose. Plays a central role in the degradation of complex lipids and the turnover of cellular membranes. Through the production of ceramides, participates in the PKC-activated salvage pathway of ceramide formation. Catalyzes the glucosylation of cholesterol, through a transglucosylation reaction where glucose is transferred from GlcCer to cholesterol. GlcCer containing mono-unsaturated fatty acids (such as beta-D-glucosyl-N-(9Z-octadecenoyl)-sphing-4-enine) are preferred as glucose donors for cholesterol glucosylation when compared with GlcCer containing same chain length of saturated fatty acids (such as beta-D-glucosyl-N-octadecanoyl-sphing-4-enine). 1. Ron, I. et al. (2005) Blood cells, molecules & diseases 35, 57-65. PMID: 159169072. Akiyama, H. et al. (2013) Biochemical and biophysical research communications 441, 838-43. PMID: 242112083. Akiyama, H. et al. (2020) The Journal of biological chemistry 295, 5257-5277. PMID: 321442044. Bannink, S. et al. (2024) Journal of lipid research 65, 100670. PMID: 393957895. Vaccaro, AM. et al. (1997) The Journal of biological chemistry 272, 16862-7. PMID: 92019936. Magalhaes, J. et al. (2016) Human molecular genetics 25, 3432-3445. PMID: 273786987. Kitatani, K. et al. (2009) The Journal of biological chemistry 284, 12972-8. PMID: 192790118. Marques, AR. et al. (2016) Journal of lipid research 57, 451-63. PMID: 267244859. Boer, DE. et al. (2021) Journal of lipid research 62, 100018. PMID: 33361282 | 
