Skip to main content
SpringerLink
Log in

Presence of glycoproteins containing the polylactosamine structure in brain and liver of GM1 gangliosidosis patients

Comparative study between clinical types I and II, using endo-β-galactosidase enzyme

  • Original Articles
  • Published:
Neurochemical Pathology

Abstract

The material derived from defective degradation of glycoproteins, which accumulates in brain and liver of a patient with GM1 gangliosidosis type I, was investigated, and the structure of the main storage compounds determined.

For comparison, brain and liver of a patient with GM1 gangliosidosis type II were also analyzed.

Analysis of the glycopeptides obtained after pronase digestion of the defatted residue indicates the storage of glycoprotein-like material in type I, but not in type II. Treatment with endo-β-galactosidase showed that the stored material containedN-acetyllactosamine repeating units.

Two major oligosaccharides, OS I and OS II, were isolated after the enzyme treatment, whose structures are: GlcNAcβ1→3 Gal (OS I) and Galβl→4GlcNAcβ1→3 Gal (OS II).

Treatment with exo-β-galactosidase transformed the trisaccharide OS II into the disaccharide OS I, indicating that the deficiency of β-galactosidase in GM1 gangliosidosis type I, but not in type II, also affects glycoprotein catabolism, leading to the accumulation of glycopeptides containing terminal β-galactosyl residues andN-acetyllactosamine repeating units.

These results indicate the severe impairment in the catabolism of glycoconjugates with β-linked galactose in type I, although this impairment is not as pronounced in type II.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  • Berra B., Di Palma S., and Brunngraber E. G. (1974) Altered levels of tissue gangliosides and glycoproteins in the infantile form of GM1 gangliosidosis.Clin. Chim. Acta 57, 301–306.

    Article  PubMed  CAS  Google Scholar 

  • Bhatti, T., Chambers, R. E., and Clamp, J. R. (1970) The gas chromatographic properties of biologically important N-acetylglucosamine derivatives, monosaccharides, disaccharides, trisaccharides, tetrasaccharides, and pentasaccharides.Biochim. Biophys. Acta 222, 339–347.

    PubMed  CAS  Google Scholar 

  • Di Palma S., Berra B., Li Y. T., and Li S. C., (1978) Storage of glycoproteins containing terminal galactose in brain of two patients with type I GM1 gangliosidosis, inPerspectives in Inherited Metabolic Diseases, vol. 1, pp. 155–164. Edi-Ermes, Milano.

    Google Scholar 

  • Dubois M., Gilles K. A., Hamilton, J. K., Rebers, P. A., and Smith F. (1956) Colorimetric determination of sugars and related substances.Anal. Chem. 28, 350–356.

    Article  CAS  Google Scholar 

  • Harris G. and MacWilliam J. C. (1954) Dipping technique for revealing neutral sugars on paper chromatography.Chem. Ind. (London) pp. 249.

  • Jarnefelt J., Rush J., Li Y. T., and Laine R. (1978) Erythroglycan, a high molecular weight glycopeptide with the repeating structure galactosyl (1→4)-2-deoxy-2-acetamido-glucosyl (1→3) comprising more than onethird of the protein bound carbohydrates of human erythrocyte stroma.J. Biol. Chem. 253, 8006–8009.

    PubMed  CAS  Google Scholar 

  • Li Y. T. and Li S. C. (1972) Degradation of complex carbohydrates, section VI, inMethods in Enzymology, vol. 28, (Ginsburg V., ed.) pp. 702–713, Academic, NY, NY.

    Google Scholar 

  • Nakagawa H., Yamada T., Chien J. L., Gardas A., Kitamikado M., Li S. C., and Li Y. T. (1980) Isolation and characterization of an endo-β-galactosidase from a new strain ofEscherichia freundii.J. Biol. Chem. 255, 5955–5959.

    PubMed  CAS  Google Scholar 

  • O’Brien J. (1983) The Gangliosidoses, in the Metabolic Basis of Inherited Disease (Stanbury J. B., Wyngaarden J. B., Fredrickson D. S., Goldstein M. D., and Brown M. D. eds) pp. 945–969, McGraw Hill, New York.

    Google Scholar 

  • Ohkura T., Yamashita K., Kobata A. (1981) Urinary oligosaccharides of GM1 gangliosidosis. Structures of oligosaccharides excreted in the urine of type I but not in the urine of type II patients.J. Biol. Chem. 256, 8485–8490.

    PubMed  CAS  Google Scholar 

  • Suzuki K. and Kamoshita S. (1969) Chemical pathology of GM1 gangliosidosis (generalized gangliosidosis).J. Neuropathol. Exp. Neurol. 28, 25–73.

    Article  PubMed  CAS  Google Scholar 

  • Tettamanti G., Bonali F., Marchesini S., and Zambotti V. (1973) A new procedure for extraction, purification, and fractionation of brain gangliosides.Biochim. Biophys. Acta 296, 160–170.

    PubMed  CAS  Google Scholar 

  • Tsay G. C., Dawson G., and Li Y. T. (1975) Structure of the glycopeptide storage material in GM1 gangliosidosis. Sequence determination with specific endo- and exo-glycosidases.Biochim. Biophys. Acta 385, 305–311.

    PubMed  CAS  Google Scholar 

  • Yamashita K., Ohkura T., Okada S., Yabuuchi H., and Kobata A. (1981) Urinary oligosaccharides of GM1-gangliosidosis. Different excretion patterns of oligosaccharides in the urine of type I and II subgroups.J. Biol. Chem. 256, 4789–4798.

    PubMed  CAS  Google Scholar 

  • Yutaka T., Okada S., Minaki K., Sugita T., and Yabuuchi H. (1975) Enzymatic study of GM1 gangliosidosis.Clin. Chim. Acta 59, 283–290.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Author notes

  1. Rita De Gasperi

    Present address: Department of Biochemistry, Tulane University, School of Medicine, New Orleans, USA

Authors and Affiliations

  1. Institute of General Physiology and Biochemistry, School of Pharmacy, University of Milano, Italy

    Bruno Berra & Silvana Rapelli

  2. Department of Pediatrics, Osaka University Hospital, Osaka, Japan

    Shintaro Okada

  3. Department of Biochemistry, Tulane University, School of Medicine, New Orleans, USA

    Su-Chen Li & Yu-Teh Li

Authors
  1. Bruno Berra
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Rita De Gasperi
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Silvana Rapelli
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Shintaro Okada
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Su-Chen Li
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Yu-Teh Li
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Berra, B., De Gasperi, R., Rapelli, S. et al. Presence of glycoproteins containing the polylactosamine structure in brain and liver of GM1 gangliosidosis patients. Neurochemical Pathology 4, 107–117 (1986). https://doi.org/10.1007/BF03160189

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF03160189

Index Entries

Search

Navigation