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Ganglioside Structure, Function, and Biomedical Potential pp 431–440Cite as

Canine GM2-Gangliosidosis: Chemical and Enzymatic Features

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Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 174))

Abstract

GM2-gangliosidosis in animals has been observed in cat, dog and swine.1–4 In 1968, McGrath et al. found a new strain of GM2-gangliosidosis in male German short hair pointer dog which had shown signs of progressive neural dysfunction. Onset of the disease was 10–11 months. Clinical signs initiated by a deterioration of his previously keen response to a whistle, included in sequence: incoordination and postual difficulties, progressive impairment of hearing and vision, psychic changes simulating idiocy, terminal convulsions and prostration. Histological observations showed typical ballooning of the cell bodies of many neurons throughout the nervous system. The intracyto-plasmic materials was Sudan black B and PAS positive. Electron microscopic examination revealed typical MCB as found in human Tay-Sachs disease.4 Clinical and pathological features in dog GM2-gangliosidosis appear to be similar to human Tay-Sachs disease. Brief biochemical features of canine GM2-gangliosidosis were previously reported by Autilio-Gambetti. The present report concerns more detail on chemical and enzymatic analysis in tissues of canine GM2-gangliosidosis.

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References

  1. J. S. O’Brien, S. Okada, M. W. Ho, D. L. Fillerup, M. L. Veath, and K. Adams, Ganglioside storage disease, Fed. Proc. 30:956 (1971).

    PubMed  Google Scholar 

  2. H. J. Baker, J. Mole, J. R. Lindsey, and R. M. Creel, Animal models of human ganglioside storage diseases, Fed. Proc. 35:1193 (1976).

    PubMed  CAS  Google Scholar 

  3. H. Bernheimer, H. and E. Karbe, Morphologische und neuro-chemische Untersuchungen von 2 Formen der amourotischen Idiotie des Hundes: Nachweis einer GM2-gangliosidose Acta Neuropathol. 16:243 (1970).

    Article  PubMed  CAS  Google Scholar 

  4. J. H. McGrath, A. M. Kelly, L. Autilio-Gambetti, and S. A. Steinberg, Nervous system: Storage diseases in dogs and cats, in: “International Encyclopedia of Psychiatry, Psychology, Psychoanalysis and Neurology,” Vol. 8, B. B. Wolman, ed., Human Sciences Press, New York (1977).

    Google Scholar 

  5. J. Folch, M. G. Lees, and G. H. Sloan Stanley, A simple method for the isolation and purification of total lipids from animal tissues, J. Biol. Chem. 226:497 (1957).

    PubMed  CAS  Google Scholar 

  6. K. Suzuki, The pattern of mammalian brain gangliosides. III. Regional and developmental differences, J. Neurochem. 11:169 (1965).

    Google Scholar 

  7. L. Svennerholm, Quantitative estimation of sialic acids. II. A colorimetric resorcinol-hydrochloric acid method, Biochim. Biophys. Acta 24:604 (1957).

    Article  PubMed  CAS  Google Scholar 

  8. S. Ando, N.-C. Chang, and R. K. Yu, High performance thin-layer chromatography and densitometric determination of brain ganglioside composition of several species, Anal. Biochem. 89:437 (1978).

    Article  PubMed  CAS  Google Scholar 

  9. Y.-T. Li, M. J. King, and S.-C. Li, Enzymic degradation of gangliosides, In: “Structure and Function of Gangliosides,” Vol. 125, p. 93, Plenum Press, New York (1980).

    Google Scholar 

  10. T. Momoi, S. Ando, and Y. Nagai, High resolution preparative column chromatographic system for gangliosides using DEAE-Sephadex and a new porous silica, Iatrobeads, Biochim. Biophys. Acta 441:488 (1976).

    PubMed  CAS  Google Scholar 

  11. Y. Z. Frohwein and S. Gatt, Isolation of N-acetyl-beta-hexosaminidase from calf brain, Biochemistry 6:2775 (1967).

    Article  PubMed  CAS  Google Scholar 

  12. C. W. Wringley, Analytical fractionation of plant and animal proteins by gel electrofocusing, J. Chromat. 36:362 (1968).

    Article  Google Scholar 

  13. D. Robinson, R. G. Price, and N. B. Dance, Separation and properties of beta-glucosidase, beta-glucuronidase and N-acetyl-beta-glucosaminidase from rat kidney, Biochem. J. 102:525 (1967).

    PubMed  CAS  Google Scholar 

  14. L. C. Cork, J. F. Munnell, M. D. Lorenz, J. V. Murphy, H. J. Baker, and M. C. Rattazzi, GM2-ganglioside lyosomal storage disease in cats with beta-hexosaminidase deficiency, Science 196:1014 (1977).

    Article  PubMed  CAS  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Pediatrics, The Tokyo Jikei University School of Medicine, Tokyo, Japan

    Yoshikatsu Eto

  2. Institute of Pathology, Case Western Reserve University, Cleveland, Ohio, USA

    Lucila Autilio-Gambetti

  3. Veterinary School, University of Pennsylvania, Philadelphia, USA

    John T. McGrath

Authors
  1. Yoshikatsu Eto
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  2. Lucila Autilio-Gambetti
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  3. John T. McGrath
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Editor information

Editors and Affiliations

  1. Albert Einstein College of Medicine, Bronx, New York, USA

    Robert W. Ledeen  & Kunihiko Suzuki  & 

  2. Yale University School of Medicine, New Haven, Connecticut, USA

    Robert K. Yu

  3. New York State Psychiatric Institute, New York, New York, USA

    Maurice M. Rapport

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© 1984 Plenum Press, New York

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Eto, Y., Autilio-Gambetti, L., McGrath, J.T. (1984). Canine GM2-Gangliosidosis: Chemical and Enzymatic Features. In: Ledeen, R.W., Yu, R.K., Rapport, M.M., Suzuki, K. (eds) Ganglioside Structure, Function, and Biomedical Potential. Advances in Experimental Medicine and Biology, vol 174. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-1200-0_36

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  • DOI: https://doi.org/10.1007/978-1-4684-1200-0_36

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4684-1202-4

  • Online ISBN: 978-1-4684-1200-0

  • eBook Packages: Springer Book Archive

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