The world's first wiki where authorship really matters (Nature Genetics, 2008). Due credit and reputation for authors. Imagine a global collaborative knowledge base for original thoughts. Search thousands of articles and collaborate with scientists around the globe.

wikigene or wiki gene protein drug chemical gene disease author authorship tracking collaborative publishing evolutionary knowledge reputation system wiki2.0 global collaboration genes proteins drugs chemicals diseases compound
Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)



Gene Review

Glb1  -  galactosidase, beta 1

Mus musculus

Synonyms: AW125515, Acid beta-galactosidase, Beta-galactosidase, Bge, Bgl, ...
Welcome! If you are familiar with the subject of this article, you can contribute to this open access knowledge base by deleting incorrect information, restructuring or completely rewriting any text. Read more.

Disease relevance of Glb1


High impact information on Glb1

  • Our results indicate that Bgl-s acts cis, controlling the expression of the structural gene located on the same chromosome [5].
  • The observed recombination frequency for the intervals between Fv-2 and Bgl-s was 6.8% [6].
  • At least 10 centimorgans of Fv-2r-linked chromosomal material originating from the C57BL/6 ancestor remains in the D2.B6-Fv-2r genome, as shown by the continued presence of C57BL/6 alleles at the flanking Kfo-1 and Bgl-s loci [6].
  • This hypothesis is consistent with the observations that rotaviruses seem to infect only gut epithelial cells, and that infant animals, whose lactase concentrations are generally higher than those of adult animals, seem more susceptible to rotavirus infections [4].
  • The lag time for secretion of newly synthesized beta-galactosidase precursor was notably longer than that for the beta-glucuronidase precursor [7].

Chemical compound and disease context of Glb1

  • GM1 gangliosidosis is a progressive neurodegenerative disease caused by deficiencies in lysosomal acid beta-galactosidase (beta-gal) and involves accumulation and storage of ganglioside GM1 and its asialo form (GA1) in brain and visceral tissues [8].

Biological context of Glb1


Anatomical context of Glb1

  • Introduction of one polymorphism, Asn517-->Asp into the C57BL cDNA, did not alter the acid beta-galactosidase activity in the transfected COS-1 cells, while introduction of Gly539-->Arg completely abolished the catalytic activity [13].
  • 3. Transfer and retention of 346J21 in breast cancer cell lines leads to growth arrest after 8-10 cell doublings, accompanied by the appearance of characteristic senescent cell morphology and senescence-associated acid beta-galactosidase activity [14].
  • MIC cells co-cultured with 14-day intestinal endoderms promoted endodermal cell adhesion and growth, and the addition of exogeneous RA enhanced epithelial cell polarization and differentiation assessed by cytokeratin and lactase immunostaining [15].
  • Correction of acid beta-galactosidase deficiency in GM1 gangliosidosis human fibroblasts by retrovirus vector-mediated gene transfer: higher efficiency of release and cross-correction by the murine enzyme [16].
  • The predicted maximal lactase and alpha-glucosidase activities expressed by enterocytes migrating over Peyer's patch follicles were about one-quarter and one-half of values found in control villi [17].

Associations of Glb1 with chemical compounds

  • A type 1 diabetes-related protein from wheat (Triticum aestivum). cDNA clone of a wheat storage globulin, Glb1, linked to islet damage [10].
  • GM1-gangliosidosis is a progressive neurological disease in humans caused by deficiency of lysosomal acid beta-galactosidase, which hydrolyses the terminal beta-galactosidic residue from ganglioside GM1 and other glycoconjugates [18].
  • The inhibition of mouse beta-galactosidase by the active-site-directed reagent N-bromoacetyl-beta-D-galactosylamine has been investigated. beta-Galactosidase from the high and low Bgs strains has identical affinity for this inhibitor [19].
  • We measured the safety factor of the mouse intestinal brush-border hydrolase maltase in series with the glucose transporter SGLT1, for comparison with previous studies of sucrase and lactase [20].
  • 2. Glucose-, fructose- and 3-O-methyl-glucose-containing diets increased sucrase and maltase but not lactase activities in mouse jejunal homogenates [21].

Other interactions of Glb1

  • These observations are contrary to the expected outcome of Mendelian autosomal recessive single gene disorders and may also be interpreted as that the acid beta-galactosidase gene functions as a modifier gene for the phenotypic expression of genetic galactosylceramidase deficiency [9].
  • The presence of the same beta-galactosidase bands in homogenates of rat kidney and small intestine as well as in neuraminidase-treated homogenates of liver and spleen suggests that the Glb-1 variants differ by one or more point mutations in the structural gene for "acid" beta-galactosidase [22].
  • No significant linkage between Glb-1 and albino (LG I), brown (LG II), or hooded (LG VI) was observed [22].

Analytical, diagnostic and therapeutic context of Glb1


  1. Generalized CNS disease and massive GM1-ganglioside accumulation in mice defective in lysosomal acid beta-galactosidase. Hahn, C.N., del Pilar Martin, M., Schröder, M., Vanier, M.T., Hara, Y., Suzuki, K., Suzuki, K., d'Azzo, A. Hum. Mol. Genet. (1997) [Pubmed]
  2. Substrate reduction reduces gangliosides in postnatal cerebrum-brainstem and cerebellum in GM1 gangliosidosis mice. Kasperzyk, J.L., d'Azzo, A., Platt, F.M., Alroy, J., Seyfried, T.N. J. Lipid Res. (2005) [Pubmed]
  3. Expression of polyoma early functions in mouse embryonal carcinoma cells depends on sequence rearrangements in the beginning of the late region. Katinka, M., Yaniv, M., Vasseur, M., Blangy, D. Cell (1980) [Pubmed]
  4. Is lactase the receptor and uncoating enzyme for infantile enteritis (rota) viruses? Holmes, I.H., Rodger, S.M., Schnagl, R.D., Ruck, B.J., Gust, I.D., Bishop, R.F., Barnes, G.L. Lancet (1976) [Pubmed]
  5. Cis-active control of mouse beta-galactosidase biosynthesis by a systemic regulatory locus. Berger, F.G., Paigen, K. Nature (1979) [Pubmed]
  6. Construction of a D2.B6-Fv-2r congenic mouse strain: nonlethality of the homozygous Fv-2' genotype. Dizik, M., Elliott, R.W., Lilly, F. J. Natl. Cancer Inst. (1981) [Pubmed]
  7. Effects of ammonia on processing and secretion of precursor and mature lysosomal enzyme from macrophages of normal and pale ear mice: evidence for two distinct pathways. Brown, J.A., Novak, E.K., Swank, R.T. J. Cell Biol. (1985) [Pubmed]
  8. Inheritance of lysosomal acid beta-galactosidase activity and gangliosides in crosses of DBA/2J and knockout mice. Hauser, E.C., Kasperzyk, J.L., d'Azzo, A., Seyfried, T.N. Biochem. Genet. (2004) [Pubmed]
  9. Paradoxical influence of acid beta-galactosidase gene dosage on phenotype of the twitcher mouse (genetic galactosylceramidase deficiency). Tohyama, J., Vanier, M.T., Suzuki, K., Ezoe, T., Matsuda, J., Suzuki, K. Hum. Mol. Genet. (2000) [Pubmed]
  10. A type 1 diabetes-related protein from wheat (Triticum aestivum). cDNA clone of a wheat storage globulin, Glb1, linked to islet damage. MacFarlane, A.J., Burghardt, K.M., Kelly, J., Simell, T., Simell, O., Altosaar, I., Scott, F.W. J. Biol. Chem. (2003) [Pubmed]
  11. Substrate specificity and distribution of acid beta-galactosidase activities in seizure-susceptible and non-susceptible strains of mice. Wheeler, D.F., Bachelard, H.S. Neurochem. Res. (1984) [Pubmed]
  12. Correlation between structural variation and activity of murine kidney beta-galactosidase: implications for genetic control. Li, I.C., Daniel, W.L. Biochem. Genet. (1976) [Pubmed]
  13. Effects of double amino-acid substitution polymorphism in acid beta-galactosidase gene in two inbred strains of mice. Hara, Y., Nishimoto, J., Suzuki, K. Biochim. Biophys. Acta (1994) [Pubmed]
  14. Functional identification of a BAC clone from 16q24 carrying a senescence gene SEN16 for breast cancer cells. Kaur, G.P., Reddy, D.E., Zimonjic, D.B., de Riel, J.K., Athwal, R.S. Oncogene (2005) [Pubmed]
  15. Mesenchyme-mediated effects of retinoic acid during rat intestinal development. Plateroti, M., Freund, J.N., Leberquier, C., Kedinger, M. J. Cell. Sci. (1997) [Pubmed]
  16. Correction of acid beta-galactosidase deficiency in GM1 gangliosidosis human fibroblasts by retrovirus vector-mediated gene transfer: higher efficiency of release and cross-correction by the murine enzyme. Sena-Esteves, M., Camp, S.M., Alroy, J., Breakefield, X.O., Kaye, E.M. Hum. Gene Ther. (2000) [Pubmed]
  17. Selective expression of brush border hydrolases by mouse Peyer's patch and jejunal villus enterocytes. Smith, M.W. J. Cell. Physiol. (1985) [Pubmed]
  18. Beta-galactosidase-deficient mouse as an animal model for GM1-gangliosidosis. Matsuda, J., Suzuki, O., Oshima, A., Ogura, A., Noguchi, Y., Yamamoto, Y., Asano, T., Takimoto, K., Sukegawa, K., Suzuki, Y., Naiki, M. Glycoconj. J. (1997) [Pubmed]
  19. Effects of the Bgs locus on mouse beta-galactosidase. Meisler, M.H. Biochem. Genet. (1976) [Pubmed]
  20. Loads, capacities and safety factors of maltase and the glucose transporter SGLT1 in mouse intestinal brush border. Lam, M.M., O'Connor, T.P., Diamond, J. J. Physiol. (Lond.) (2002) [Pubmed]
  21. Sugar-dependent selective induction of mouse jejunal disaccharidase activities. Collins, A.J., James, P.S., Smith, M.W. J. Physiol. (Lond.) (1989) [Pubmed]
  22. Genetically controlled variation of "acid" beta-galactosidase detected in Rattus norvegicus by isoelectric focusing. Douglas, T.C., Kimmel, K.A., Dawson, P.E. Genetics (1982) [Pubmed]
  23. Molecular cloning of mouse acid beta-galactosidase cDNA: sequence, expression of catalytic activity and comparison with the human enzyme. Nanba, E., Suzuki, K. Biochem. Biophys. Res. Commun. (1990) [Pubmed]
  24. Induction of endogenous beta-galactosidase by ionizing radiation complicates the analysis of p53-LacZ transgenic mice. Coates, P.J., Lorimore, S.A., Rigat, B.A., Lane, D.P., Wright, E.G. Oncogene (2001) [Pubmed]
  25. Effects of fecal microorganisms and their chloroform-resistant variants derived from mice, rats, and humans on immunological and physiological characteristics of the intestines of ex-germfree mice. Okada, Y., Setoyama, H., Matsumoto, S., Imaoka, A., Nanno, M., Kawaguchi, M., Umesaki, Y. Infect. Immun. (1994) [Pubmed]
WikiGenes - Universities