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 (PubMed:9201993, PubMed:24211208, PubMed:15916907, PubMed:32144204). Plays a central role in the degradation of complex lipids and the turnover of cellular membranes (PubMed:27378698). Through the production of ceramides, participates in the PKC-activated salvage pathway of ceramide formation (PubMed:19279011). Catalyzes the glucosylation of cholesterol, through a transglucosylation reaction where glucose is transferred from GlcCer to cholesterol (PubMed:24211208, PubMed:26724485, PubMed:32144204). 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) (PubMed:24211208). Under specific conditions, may alternatively catalyze the reverse reaction, transferring glucose from cholesteryl 3-beta-D-glucoside to ceramide (PubMed:26724485) (Probable). Can also hydrolyze cholesteryl 3-beta-D-glucoside producing glucose and cholesterol (PubMed:24211208, PubMed:26724485). Catalyzes the hydrolysis of galactosylceramides/GalCers (such as beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine), as well as the transfer of galactose between GalCers and cholesterol in vitro, but with lower activity than with GlcCers (PubMed:32144204). Contrary to GlcCer and GalCer, xylosylceramide/XylCer (such as beta-D-xyosyl-(1<->1')-N-acylsphing-4-enine) is not a good substrate for hydrolysis, however it is a good xylose donor for transxylosylation activity to form cholesteryl 3-beta-D-xyloside (PubMed:33361282). a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + H2O = an N-acylsphing-4-enine + D-glucose a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine + H2O = an N-acylsphing-4-enine + D-galactose cholesteryl 3-beta-D-glucoside + H2O = cholesterol + D-glucose a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = an N-acylsphing-4-enine + cholesteryl 3-beta-D-glucoside beta-D-glucosyl-N-(9Z-octadecenoyl)-sphing-4E-enine + cholesterol = cholesteryl 3-beta-D-glucoside + N-(9Z-octadecenoyl)-sphing-4-enine beta-D-glucosyl-(1<->1')-N-hexadecanoylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + N-hexadecanoylsphing-4-enine beta-D-glucosyl-N-octanoylsphing-4E-enine + cholesterol = cholesteryl 3-beta-D-glucoside + N-octanoylsphing-4-enine beta-D-glucosyl-N-dodecanoylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + N-dodecanoylsphing-4-enine beta-D-glucosyl-(1<->1)-N-octadecanoylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + N-octadecanoylsphing-4-enine beta-D-glucosyl-(1<->1')-N-(15Z-tetracosenoyl)-sphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + N-(15Z-tetracosenoyl)-sphing-4-enine a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = an N-acylsphing-4-enine + cholesteryl 3-beta-D-galactoside 1-(beta-D-galactosyl)-N-dodecanoylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-galactoside + N-dodecanoylsphing-4-enine a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = an N-acylsphing-4-enine + cholesteryl 3-beta-D-xyloside beta-D-xylosyl-(1<->1')-N-(9Z-octadecenoyl)-sphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + N-(9Z-octadecenoyl)-sphing-4-enine Synergistically activated by saposin-A and saposin-C, two saposin peptides produced by proteolytic processing of prosaposin/PSAP (PubMed:9201993). Saposin-C activates GBA through its recruitment to membranes (PubMed:10781797, PubMed:9201993). The membrane structure and composition in anionic phospholipids are also important for the activation (PubMed:9201993, PubMed:10781797). Activated by PKC in the salvage pathway of ceramide formation (PubMed:19279011). Inhibited by conduritol B epoxide/CBE (PubMed:24211208, PubMed:26724485). Optimum pH is 5.3. Optimum temperature is 43 degrees Celsius. Steroid metabolism; cholesterol metabolism. Sphingolipid metabolism. Interacts with saposin-C (PubMed:10781797). Interacts with SCARB2 (PubMed:18022370). Interacts with TCP1 (PubMed:21098288). May interact with SNCA; may inhibit the glucosylceramidase activity (PubMed:23266198). Interacts with GRN; this interaction prevents aggregation of GBA-SCARB2 complex via interaction with HSPA1A upon stress (PubMed:27789271). Interaction with saposin-C promotes membrane association (PubMed:10781797). Targeting to lysosomes occurs through an alternative MPR-independent mechanism via SCARB2 (PubMed:18022370). The disease is caused by variants affecting the gene represented in this entry. The disease is caused by variants affecting the gene represented in this entry. The disease is caused by variants affecting the gene represented in this entry. The disease is caused by variants affecting the gene represented in this entry. The disease is caused by variants affecting the gene represented in this entry. The disease is caused by variants affecting the gene represented in this entry. Perinatal lethal Gaucher disease is associated with non-immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders. Disease susceptibility may be associated with variants affecting the gene represented in this entry. Available under the names Ceredase and Cerezyme (Genzyme). Used to treat Gaucher disease. Major isoform. Produced by alternative initiation from a downstream AUG. Two to three times less protein is produced from this downstream AUG. Produced by alternative splicing. Belongs to the glycosyl hydrolase 30 family. Clinical information on Ceredase Clinical information on Cerezyme