Summary

Name GBA
Full Name Lysosomal acid glucosylceramidase
Synonyms Acid beta-glucosidase, Alglucerase, Beta-glucocerebrosidase, Beta-GC, D-glucosyl-N-acylsphingosine glucohydrolase, Imiglucerase | GC, GLUC
Primary ID P04062
Links - -
Type protein
Relations 3
Function Glucosylceramidase that catalyzes, within the lysosomal compartment, the hydrolysis of glucosylceramide/GlcCer into free ceramide and glucose (PubMed:9201993, PubMed:24211208, PubMed:15916907). Thereby, 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). Also plays a role in cholesterol metabolism (PubMed:24211208, PubMed:26724485). May either catalyze the glucosylation of cholesterol, through a transglucosylation reaction that transfers glucose from glucosylceramide to cholesterol (PubMed:24211208, PubMed:26724485). The short chain saturated C8:0-GlcCer and the mono-unsaturated C18:0-GlcCer being the most effective glucose donors for that transglucosylation reaction (PubMed:24211208). Under specific conditions, may alternatively catalyze the reverse reaction, transferring glucose from cholesteryl-beta-D-glucoside to ceramide (PubMed:26724485). Finally, may also hydrolyze cholesteryl-beta-D-glucoside to produce D-glucose and cholesterol (PubMed:24211208, PubMed:26724485).

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Relations

Regulator Mechanism target score
+ up-regulates quantity by expression img/indirect-activation.png transcriptional regulation GBA 0.2
Publications: 1
+ up-regulates quantity by expression img/direct-activation.png transcriptional regulation GBA 0.2
Publications: 1
+ up-regulates quantity by expression img/direct-activation.png transcriptional regulation GBA 0.332
Publications: 1