Glucosylceramidase beta 3 (GBA3)

Glucosylceramidase beta 3 (GBA3) is a cytosolic enzyme with broad substrate specificity that hydrolyzes various glycosides, including galactosylceramides, glucosylsphingosines, and dietary glycosides such as flavonoid and cyanogenic glycosides[1][2][3]. It is expressed predominantly in the liver, kidney, intestine, and spleen[1][6]. Unlike lysosomal glucosylceramidase (GBA), whose deficiency causes Gaucher's disease, GBA3 is not known to directly cause human disease but is implicated in non-lysosomal pathways of glycosylceramide metabolism and detoxification of dietary plant glycosides[2][3][4]. The enzyme functions optimally at neutral pH. GBA3 is polymorphic, with the most common allele encoding the functional protein, but pseudogenic alleles are present in the population[1][6]. While possessing significant glycosylceramidase activity in vitro, its physiological relevance in vivo remains uncertain[1][2].

Other names

Cytosolic beta-glucosidaseCytosolic beta-glucosidase-like protein 1CBGL1CBGGLUCKlotho-related protein (KLrP)Cytosolic galactosylceramidaseCytosolic glucosylceramidaseCytosolic glycosylceramidaseGlucosidase, beta, acid 3 (cytosolic)