Advertisement

Studies on the Pathogenesis of Krabbe’s Leukodystrophy: Cellular Reaction of the Brain to Exogenous Galactosylsphingosine, Monogalactosyl Diglyceride, and Lactosylceramide

  • Kinuko Suzuki
  • Harumi Tanaka
  • Kunihiko Suzuki
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 68)

Abstract

Krabbe’s leukodystrophy or globoid cell leukodystrophy (GLD) is an autosomal recessive neurological disorder of infancy, affecting the central as well as peripheral nervous system. Tonic seizures, generalized convulsions, spastic quadriplegia, optic atrophy and deafness with rapid clinical progression are major symptoms of the patient with GLD.

Keywords

Cytoplasmic Inclusion Neuronal Cytoplasmic Inclusion Globoid Cell Leukodystrophy Spastic Quadriplegia Acta Neuropath 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Andrews, J.M. and Menkes, J.H.: Ultrastructure of experimentally produced globoid cells in the rat. Exper. Neurol. 29: 483, 1970.CrossRefGoogle Scholar
  2. 2.
    Austin, J.H.: Studies in globoid (Krabbe) leukodystrophy. II. Controlled thin-layer chromatographic studies of globoid body fraction in seven patients. J. Neurochem. 10: 921, 1963.PubMedCrossRefGoogle Scholar
  3. 3.
    Austin, J.H. and Lehfeldt, D.: Studies in globoid (Krabbe) leukodystrophy. III. Significance of experimentally-produced globoid-like elements in rat white matter and spleen. J. Neuropathol. Exp. Neurol. 24: 265, 1965.PubMedCrossRefGoogle Scholar
  4. 4.
    D’Agostino, A.N., Sayre, G.P. and Hagles, A.B.: Krabbe’s Disease. Arch. Neurol. (Chicago) 8: 82, 1963.CrossRefGoogle Scholar
  5. 5.
    Gleisen, C.A., Bay, W.W., Dukes, T.W., Brown, R.S., Read, W.K. and Pierce, K.K.: Study of chloroquine toxicity and drug-induced cerebrospinal lipodystrophy in swine, Amer. J. Path. 53: 27, 1968.Google Scholar
  6. 6.
    Lüllmann-Rauch, R.: Lipidosis-like alterations in spinal cord and cerebellar cortex of rats treated with chlorphentermine or tricyclic antidepressants. Acta Neuropath. 29: 237, 1974.PubMedCrossRefGoogle Scholar
  7. 7.
    Miyatake, T. and Suzuki, K.: Galactosyl sphingosine galactosyl hydrolase: Partial purification and properties of the enzyme in rat brain. J. Biol. Chem. 247: 5398, 1972.PubMedGoogle Scholar
  8. 8.
    Miyatake, T. and Suzuki, K.: Globoid cell leukodystrophy: Additional deficiency of psychosine galactosidase. Biochem. Biophys. Res. Commun. 48: 538, 1972.CrossRefGoogle Scholar
  9. 9.
    Miyatake, T. and Suzuki, K.: Galactosyl sphingosine galactosyl hydrolase in rat brain: Probable identity with galactosylceramide galactosyl hydrolase. J. Neurochem. 22: 231, 1974.PubMedCrossRefGoogle Scholar
  10. 10.
    Olsson, R., Sourander, P. and Svennerholm, L.: Experimental studies on the pathogenesis of leucodystrophies. I. The effect of intracerebrally injected sphingolipids in the rat’s brain. Acta Neuropath. 6:153, 1966.PubMedCrossRefGoogle Scholar
  11. 11.
    Schutta, H.S. and Neville, H.E.: Effects of cholesterol biosynthesis inhibitors on the nervous system. Lab. Invest. 19: 487, 1968.PubMedGoogle Scholar
  12. 12.
    Suzuki, K.: Ultrastructural study of experimental globoid cells. Lab. Invest. 23: 612, 1970.PubMedGoogle Scholar
  13. 13.
    Suzuki, K. and Suzuki, Y.: Globoid cell leucodystrophy (Krabbe’s disease); deficiency of galactocerebroside β-galactosidase. Proc. Nat. Acad. Sci. U.S.A. 66: 302, 1970.CrossRefGoogle Scholar
  14. 14.
    Suzuki, K., Zagoren, J.C., Gonatas, J., and Suzuki, K.: Ultrastructural study of neuronal cytoplasmic inclusions produced by hypocholesterolemic drug AY 9944. Acta Neuropath. (Berl.) 26: 185, 1973.CrossRefGoogle Scholar
  15. 15.
    Tanaka, H. and Suzuki, K.: Lactosylceramide β-galactosidase in human sphingolipidoses: Evidence for two genetically distinct enzymes. J. Biol. Chem. 250: 2324, 1975.PubMedGoogle Scholar
  16. 16.
    Vanier, M.T. and Svennerholm, L.: Chemical pathology of Krabbe’s Disease. III. Ceramide-hexosides and gangliosides of brain. Acta Paediatr. Scand. 64: 641, 1975.PubMedCrossRefGoogle Scholar
  17. 17.
    Wenger, D.A., Sattler, M. and Markey, S.P.: Deficiency of monogalactosyl diglyceride β-galactosidase activity in Krabbe’s disease. Biochem. Biophys. Res. Commun. 53: 680, 1973.PubMedCrossRefGoogle Scholar
  18. 18.
    Wenger, D.A.: Studies on galactosylceramide and lactosylceramide β-galactosidase. Chem. Phys. Lipids 13: 327, 1974.PubMedCrossRefGoogle Scholar
  19. 19.
    Wenger, D.A., Sattler, M. and Hiatt, W.: Globoid cell leukodystrophy: Deficiency of lactosyl ceramide beta-galactosidase. Proc. Nat. Acad. Sci. 71: 854, 1974.PubMedCrossRefGoogle Scholar
  20. 20.
    Yunis, E.J. and Lee, R.E.: The ultrastructure of globoid (Krabbe) leukodystrophy. Lab. Invest. 21: 415, 1969.Google Scholar
  21. 21.
    Yunis, E. and Lee, R.E.: Further observations on the fine structure of globoid leukodystrophy. Human Pathology 3: 371, 1972.PubMedCrossRefGoogle Scholar

Copyright information

© Plenum Press, New York 1976

Authors and Affiliations

  • Kinuko Suzuki
    • 1
    • 2
    • 3
  • Harumi Tanaka
    • 1
    • 2
    • 3
  • Kunihiko Suzuki
    • 1
    • 2
    • 3
  1. 1.Department of Pathology (Neuropathology)Albert Einstein College of MedicineBronxUSA
  2. 2.The Saul S. Korey Department of NeurologyAlbert Einstein College of MedicineBronxUSA
  3. 3.Department of Neuroscience and Rose F. Kennedy Center for Research in Mental Retardation and Human DevelopmentAlbert Einstein College of MedicineBronxUSA

Personalised recommendations