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Gene Review

GBA3  -  glucosidase, beta, acid 3 (gene/pseudogene)

Homo sapiens

Synonyms: CBG, CBGL1, Cytosolic beta-glucosidase, Cytosolic beta-glucosidase-like protein 1, GLUC, ...
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Disease relevance of GBA3

  • Mutations in the gene encoding cytosolic beta-glucosidase in Gaucher disease [1].
  • Experimental patients practiced an algorithm for adjustment of insulin dosage based on daily prebreakfast capillary blood glucose (CBG) measurements and any symptomatic hypoglycemia [2].
  • To estimate the frequency of an early-morning glucose rise (EMR) in relatively unselected children with insulin-dependent diabetes mellitus (IDDM), we assessed capillary blood glucose (CBG) at midsleep (0200-0430) and prebreakfast (0700-0800) in 97 children with diabetes at camp [3].
  • We conclude that serum CBG is elevated before transmural intestinal injury in this model and may have utility as an early marker of ischemia in patients at risk for NEC [4].
  • Serum cytosolic beta-glucosidase activity in a rat model of necrotizing enterocolitis [4].

High impact information on GBA3

  • Beta-Glucosides of pyridoxine (a) are prevalent in plant-derived foods, (b) contribute to human nutrition as partially available sources of vitamin B6, (c) undergo partial hydrolysis by a novel mammalian cytosolic beta-glucosidase, and (d) exert a weak antagonistic effect on the utilization of free pyridoxine [5].
  • No significant differences in the degree of metabolic control achieved, as measured by mean fasting, preprandial, and postprandial capillary blood glucose (CBG), M values, glycosylated hemoglobin A1 concentration, cholesterol and triglyceride levels were seen between MSI and CSII in the sixteen patients who completed the study [6].
  • Taken together with the three-dimensional model, these mutation studies identified the amino-acid residues in the aglycone-binding subsite of hCBG that are essential for flavonoid glucoside binding and catalysis [7].
  • Human cytosolic beta-glucosidase (hCBG) is a xenobiotic-metabolizing enzyme that hydrolyses certain flavonoid glucosides, with specificity depending on the aglycone moiety, the type of sugar and the linkage between them [7].
  • Based on the substrate specificity of the human enzymes, sequence alignment of family 1 enzymes and analysis of the hCBG structural model, we selected and mutated putative substrate (aglycone) binding site residues [7].

Chemical compound and disease context of GBA3

  • Diabetic keto-acidosis (DKA) requires prompt diagnosis and all patients arriving emergency with dehydration, shock, coma, severe respiratory difficulty and evidence of any major illness should be tested for capillary blood glucose (CBG) and urinary ketones urgently not to miss DKA [8].

Biological context of GBA3


Anatomical context of GBA3


Associations of GBA3 with chemical compounds

  • Cytosolic beta-glucosidase (EC from mammalian liver is a member of the family 1 glycoside hydrolases and is known for its ability to hydrolyse a range of beta-D-glycosides, including beta-D-glucoside and beta-D-galactoside [15].
  • The recombinant CBG (reCBG) was purified from the supernatant using a single chromatography step and was shown to be similar to the native enzyme isolated from human liver in terms of physical properties and specific activity towards 4-nitrophenyl-beta-D-glucoside [9].
  • In addition, the second-generation fructosamine assay results demonstrated a significant decrease at each week of study, as did the average CBG [16].
  • The implications of galactosylsphingosine inhibition of cytosolic beta-glucosidase and the possible role of the enzyme in glycosphingolipid metabolism are discussed [17].
  • Figure Stereoscopic view of the ribbon diagram of hCBG complexed with cortisol [18].

Other interactions of GBA3


Analytical, diagnostic and therapeutic context of GBA3

  • Testing all with OGTT was the most effective strategy, but the CBG test and risk assessment questionnaire were the most efficient [19].
  • Histopathologic injury was categorized as either no or minimal injury or mural necrosis by two masked investigators and CBG activity was measured by ELISA [4].
  • OBJECTIVES: To develop an algorithm to standardize capillary blood glucose (CBG) testing in nursing home and rehabilitation patients [20].
  • In addition, the observation that the broad-specificity, cytosolic beta-glucosidase was inhibited by hydrophobic glycosphingolipids prompted the definition of a revised purification procedure which took advantage of hydrophobic affinity chromatography [17].
  • Expression, purification, characterization and crystallization of a recombinant human cytosolic beta-glucosidase produced in insect cells [21].


  1. Mutations in the gene encoding cytosolic beta-glucosidase in Gaucher disease. Beutler, E., Beutler, L., West, C. J. Lab. Clin. Med. (2004) [Pubmed]
  2. Feasibility of adjustment of insulin dose by insulin-requiring type II diabetic patients. Floyd, J.C., Funnell, M.M., Kazi, I., Templeton, C. Diabetes Care (1990) [Pubmed]
  3. Frequency of early-morning rise in blood glucose in children with diabetes at camp. Duncan, B.B., Schmidt, M.I., Ellis, G.J., Davis, C.E., Heiss, G. Diabetes Care (1988) [Pubmed]
  4. Serum cytosolic beta-glucosidase activity in a rat model of necrotizing enterocolitis. Dimmitt, R.A., Glew, R., Colby, C., Brindle, M., Skarsgard, E., Moss, R.L. Pediatr. Res. (2003) [Pubmed]
  5. Nutritional Properties and significance of vitamin glycosides. Gregory, J.F. Annu. Rev. Nutr. (1998) [Pubmed]
  6. Comparison between continuous subcutaneous insulin infusion and multiple injections of insulin. A one-year prospective study. Schiffrin, A., Belmonte, M.M. Diabetes (1982) [Pubmed]
  7. Substrate (aglycone) specificity of human cytosolic beta-glucosidase. Berrin, J.G., Czjzek, M., Kroon, P.A., McLauchlan, W.R., Puigserver, A., Williamson, G., Juge, N. Biochem. J. (2003) [Pubmed]
  8. Metabolic emergencies in diabetes. Mukherjee, A.K., Chowdhury, M.B. Journal of the Indian Medical Association. (2006) [Pubmed]
  9. Functional expression of human liver cytosolic beta-glucosidase in Pichia pastoris. Insights into its role in the metabolism of dietary glucosides. Berrin, J.G., McLauchlan, W.R., Needs, P., Williamson, G., Puigserver, A., Kroon, P.A., Juge, N. Eur. J. Biochem. (2002) [Pubmed]
  10. Kinetic analysis of the interaction of alkyl glycosides with two human beta-glucosidases. Gopalan, V., Daniels, L.B., Glew, R.H., Claeyssens, M. Biochem. J. (1989) [Pubmed]
  11. Gene cloning and characterization of a novel cellulose-binding beta-glucosidase from Phanerochaete chrysosporium. Li, B., Renganathan, V. Appl. Environ. Microbiol. (1998) [Pubmed]
  12. Molecular cloning and expression of a novel klotho-related protein. Yahata, K., Mori, K., Arai, H., Koide, S., Ogawa, Y., Mukoyama, M., Sugawara, A., Ozaki, S., Tanaka, I., Nabeshima, Y., Nakao, K. J. Mol. Med. (2000) [Pubmed]
  13. Variable expression of leukocyte cytosolic broad-specificity beta-glucosidase activity. Forsyth, G.W., Romero, K.M., Alverson, J., VanderJagt, D.J., Glew, R.H. Clin. Chim. Acta (1993) [Pubmed]
  14. The crystal structure of a cyanogenic beta-glucosidase from white clover, a family 1 glycosyl hydrolase. Barrett, T., Suresh, C.G., Tolley, S.P., Dodson, E.J., Hughes, M.A. Structure (1995) [Pubmed]
  15. Cloning and characterization of human liver cytosolic beta-glycosidase. de Graaf, M., van Veen, I.C., van der Meulen-Muileman, I.H., Gerritsen, W.R., Pinedo, H.M., Haisma, H.J. Biochem. J. (2001) [Pubmed]
  16. Clinical validation of a second-generation fructosamine assay. Cefalu, W.T., Bell-Farrow, A.D., Petty, M., Izlar, C., Smith, J.A. Clin. Chem. (1991) [Pubmed]
  17. Galactosylsphingosine inhibition of the broad-specificity cytosolic beta-glucosidase of human liver. LaMarco, K.L., Glew, R.H. Arch. Biochem. Biophys. (1985) [Pubmed]
  18. Homology model of human corticosteroid binding globulin: a study of its steroid binding ability and a plausible mechanism of steroid hormone release at the site of inflammation. Dey, R., Roychowdhury, P. Journal of molecular modeling (Online) (2003) [Pubmed]
  19. Costs of screening for pre-diabetes among US adults: a comparison of different screening strategies. Zhang, P., Engelgau, M.M., Valdez, R., Benjamin, S.M., Cadwell, B., Narayan, K.M. Diabetes Care (2003) [Pubmed]
  20. Development of a protocol for capillary blood glucose testing in nursing home and rehabilitation settings. Mader, S.L., Fuglee, K.A., Allen, D.S., Werner, L.R., Wanlass, W.A., Pagel, K.J., Beliel, K.L., McEuen, J.A., Stephens, E.A., Allison, N.L., McWhorter, K.A., Vandling, J.E. Journal of the American Geriatrics Society. (2006) [Pubmed]
  21. Expression, purification, characterization and crystallization of a recombinant human cytosolic beta-glucosidase produced in insect cells. Fleury, D., Domaingue, P., Gillard, C., Touitou, R., Mollat, P. Protein Expr. Purif. (2007) [Pubmed]
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