The Specificity of β-Galactosidase in the Degradation of Gangliosides

  • Kunihiko Suzuki
  • Harumi Tanaka
  • Tatsuhiro Yamanaka
  • Olga Van Damme
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 125)


Degradation of gangliosides both in vivo and in vitro appears to proceed by sequential hydrolysis of the terminal carbohydrate residues, rather than by removal of oligosaccharides of two or more sugars or by initial de-acylation. These catabolic steps are catalyzed by lysosomal acidic glycosidases. Within the degradative pathways of known gangliosides, there are four compounds which possess a β-galactosyl residue at the non-reducing terminal.


White Matter Substrate Specificity Sodium Taurocholate Globoid Cell Leukodystrophy Systemic Organ Involvement 


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  1. BEN-YOSEPH, Y., HUNGERFORD, M. & NADLER, H. L. (1978): The nature of mutation in Krabee’s disease. Am. J. Human Genet. 30, 644–652.Google Scholar
  2. BEN-YOSEPH, Y., SHAPIRA, E., EDELMAN, D., BURTON, B. K. & NADLER, H. (1977): Purification and properties of neutral 8-galactosidases from human liver. Arch. Biochem. Biophys. 184, 373–380.Google Scholar
  3. BURTON, B. K., BEN-YOSEPH, Y. & NADLER, H. L. (1978): Lactosylceramidosis: Deficient activity of neutral 8-galactosidase in liver and cultured fibroblasts? Clin. Chim. Acta 88, 483–493.Google Scholar
  4. ETO, Y. & SUZUKI, K. (1971): Brain sphingoglycolipids in Krabbe’s globoid cell leukodystrophy. J. Neurochem. 18, 503–511.PubMedCrossRefGoogle Scholar
  5. MACBRINN, M. C., OKADA, S., HO, M. W., HU, C. C. & O’BRIEN, J. S. (1969): Generalized gangliosidosis: Impaired cleavage of galactose from a mucopolysaccharide and a glycoprotein. Science 163, 946–947.PubMedCrossRefGoogle Scholar
  6. MILLER, A. L., FROST, R. G. & O’BRIEN, J. S. (1977): Purified human liver acid B-D-galactosidase possessing activity towards GM1- ganglioside and lactosylceramide. Biochem. J. 165, 591–594.Google Scholar
  7. MIYATAKE, T. & SUZUKI, K. (1972): Globoid cell leukodystrophy: Additional deficiency of psychosine galactosidase. Biochem. Biophys. Res. Commun. 48, 538–543.CrossRefGoogle Scholar
  8. NISHIMURA, K. & AMANO, R. (1976): Partial purification and properties of porcine thymus lactosylceramide 8-galactosidase. J. Biochem. 80, 209–215.PubMedGoogle Scholar
  9. NORDEN, A. G. W., TENNANT, L. L. & O’BRIEN, J. S. (1974): GM11ganglioside 8-galactosidase A: Purification and studies Ut the enzyme from human liver. J. Biol. Chem. 249, 7969–7976.Google Scholar
  10. O’BRIEN, J. S. (1975): Molecular genetics of GM1–8-galactosidase. Clin. Genet. 8, 303–313.PubMedCrossRefGoogle Scholar
  11. OKADA, S. & O’BRIEN, J. S. (1968): Generalized gangliosidosis: 8-galactosidase deficiency. Science 160, 1002–1004.PubMedCrossRefGoogle Scholar
  12. SUZUKI, K. (1968): Cerebral G -gangliosidosis: Chemical pathology of visceral organs. ScienM 159, 1471–1472.CrossRefGoogle Scholar
  13. SUZUKI, K. & CHEN, G. C. (1967): Brain ceramide hexosides in Tay-Sachs disease and generalized gangliosidosis (Gml-gangliosidosis) J. Lipid Res. 8, 105–113.PubMedGoogle Scholar
  14. SUZUKI, K. & SUZUKI, Y. (1970): Globoid cell leukodystrophy (Krabbe’s disease): Deficency of galactocerebroside 8-galactosidase. Proc. Nat. Acad. Sci., U.S.A. 66, 302–309.PubMedCrossRefGoogle Scholar
  15. SUZUKI, Y. & SUZUKI, K. (1971): Krabbe’s globoid cell leukodystrophy: Deficiency of galactocerebrosidase in serum, leukocytes and fibroblasts. Science 171, 73–75.PubMedCrossRefGoogle Scholar
  16. SUZUKI, Y. & SUZUKI, K. (1974a): Glycosphingolipid ß-galactosidases II. Electrofocusing characterization of the enzymes in human globoid cell leukodystrophy (Krabbe’s disease). J. Biol. Chem. 249, 2105–2108.Google Scholar
  17. SUZUKI, Y. & SUZUKI, K. (1974b): Glycosphingolipid ß-galactosidases IV. Electrofocusing characterization in GM1-gangliosidosis. J. Biol. Chem. 249, 2113–2117.Google Scholar
  18. SUZUKI, K. & SUZUKI, Y. (1978): Galactosylceramide lipidosis: Globoid cell leukodystrophy (Krabbe’s disease), in “The Metabolic Basis of Inherited Disease”, STANBURY, J. B., WYNGAARDEN, J. B. & FREDRICKSON, D. S., Eds., McGraw-Hill, ( New York ), pp. 747–769.Google Scholar
  19. SUZUKI, K., SUZUKI, K. & KAMOSHITA, S. (1969): Chemical pathology of GM7-gangliosidosis (generalized gangliosidosis). J. Neuropath. Exp. 1Qeurol. 28, 25–73.Google Scholar
  20. SVENNERHOLM, L., HAKANSSON, G. & VANIER, M.-T. (1975): Chemical pathology of Krabbe’s disease IV. Studies of galactosyl ceramide and lactosylceramide ß-galactosidases in brain, white blood cells and amniotic fluid cells. Acta Paediat. Scand. 64, 649–656.Google Scholar
  21. TANAKA, H. & SUZUKI, K. (1975): Lactosylceramide ß-galactosidase in human sphingolipidoses: Evidence for two genetically distinct enzymes. J. Biol. Chem. 250, 2324–2332.Google Scholar
  22. TANAKA H. & SUZUKI, K. (1976): Specificities of the two genetically distinct ß-galactosidases in human sphingolipidoses. Arch. Biochem. Biophys. 175, 332–340.Google Scholar
  23. TANAKA, H. & SUZUKI, K. (1977a): Lactosylceramidase assays for diagnosis of globoid cell leukodystrophy and G -gangliosidosis. Clin. Chim. Acta 75, 267–274.Google Scholar
  24. TANAKA, H. & SUZUKI, K. (1977b): Substrate specificities of the two genetically distinct human brain S-galactosidases. Brain Res. 122, 325–335.PubMedCrossRefGoogle Scholar
  25. TANAKA, H. & SUZUKI, K. (1978): Globoid cell leukodystrophy ( Krabbe’s disease ): Metabolic studies with cultured fibroblasts. J. Neural. Sci. 38, 409–419.Google Scholar
  26. TANAKA, H., MEISLER, M. & SUZUKI, K. (1975): Activity of human hepatic S-galactosidase toward natural glycosphingolipid substrates. Biochim. Biophys. Acta 398, 452–463.Google Scholar
  27. VANIER, M.-T. & SVENNERHOLM, L. (1975): Chemical pathology of Krabbe’s disease III. Ceramide hexosides and gangliosides of brain. Acta Paediat. Scand. 64, 641–648.Google Scholar
  28. VANIER, M.-T. & SVENNERHOLM, L. (1976): Chemical pathology of Krabbe disease: The occurrence of psychosine and other neutral sphingoglycolipids. in “Current Trends in Sphingolipidoses and Allied Disorders”, VOLK, B. W. & SCHNECK, L., Eds., Plenum (new York), pp. 115–126.Google Scholar
  29. WENGER, D. A., SATTLER, M. & MARKEY, S. P. (1973): Deficiency of monogalactosyl diglyceride S-galactosidase activity in Krabbe’s disease. Biochem. Biophys. Res. Commun. 53, 680–685.Google Scholar
  30. WENGER, D. A., SATTLER, M. & HIATT, W. (1974): Globoid cell leukodystrophy: Deficiency of lactosyl ceramide beta-galactosidase. Proc. Nat. Acad. Sci., U.S.A. 71, 854–857.Google Scholar
  31. WENGER, D. A., SATTLER, M., CLARK, C., TANAKA, H., SUZUKI, K. & DAWSON, G. (1975): Lactosyl ceramidosis: Normal activity for two lactosyl ceramide S-galactosidases. Science 188, 1310–1312.Google Scholar

Copyright information

© Plenum Press, New York 1980

Authors and Affiliations

  • Kunihiko Suzuki
    • 1
  • Harumi Tanaka
    • 1
  • Tatsuhiro Yamanaka
    • 1
  • Olga Van Damme
    • 1
  1. 1.The Saul R. Korey Department of Neurology, Department of Neuroscience, and the Rose F. Kennedy Center for Research in Mental Retardation and Human DevelopmentAlbert Einstein College of MedicineBronxUSA

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