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) . Under specific conditions, may alternatively catalyze the reverse reaction, transferring glucose from cholesteryl 3-beta-D-glucoside to ceramide (Probable) . Can also hydrolyze cholesteryl 3-beta-D-glucoside producing glucose and cholesterol . 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 . 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 . .

p04062

Lysosomal acid glucosylceramidase (Lysosomal acid GCase) (EC 3.2.1.45) (Acid beta-glucosidase) (Alglucerase) (Beta-glucocerebrosidase) (Beta-GC) (Beta-glucosylceramidase 1) (Cholesterol glucosyltransferase) (SGTase) (EC 2.4.1.-) (Cholesteryl-beta-glucosidase) (EC 3.2.1.-) (D-glucosyl-N-acylsphingosine glucohydrolase) (Glucosylceramidase beta 1) (Imiglucerase) (Lysosomal cholesterol glycosyltransferase) (Lysosomal galactosylceramidase) (EC 3.2.1.46) (Lysosomal glycosylceramidase),GBA1 GBA GC GLUC,Lysosomal acid glucosylceramidase, Lysosomal acid GCase, 3.2.1.45, Acid beta-glucosidase, Alglucerase, Beta-glucocerebrosidase, Beta-GC, Beta-glucosylceramidase 1, Cholesterol glucosyltransferase, SGTase, 2.4.1.-, Cholesteryl-beta-glucosidase, 3.2.1.-, D-glucosyl-N-acylsphingosine glucohydrolase, Glucosylceramidase beta 1, Imiglucerase, Lysosomal cholesterol glycosyltransferase, Lysosomal galactosylceramidase, 3.2.1.46, Lysosomal glycosylceramidase, GBA1, GBA, GC, GLUC

Human

WB,IHC,ELISA

WB 1:500-1:2000
IHC 1:50-1:300
ELISA 1:5000-1:20000

60kD

Rabbit

Recombinant Human GBA (Ala40-Gln536).

Polyclonal

IgG

Purified by antigen affinity column.

0.01M PBS, pH 7.4, 50% Glycerol, 0.05% Proclin 300.

Use a manual defrost freezer and avoid repeated freeze-thaw cycles. Store at 4℃ short term (1-2 weeks). Store at -20℃ 12 months.