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.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
Andrews, J.M. and Menkes, J.H.: Ultrastructure of experimentally produced globoid cells in the rat. Exper. Neurol. 29: 483, 1970.
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.
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.
D’Agostino, A.N., Sayre, G.P. and Hagles, A.B.: Krabbe’s Disease. Arch. Neurol. (Chicago) 8: 82, 1963.
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.
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.
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.
Miyatake, T. and Suzuki, K.: Globoid cell leukodystrophy: Additional deficiency of psychosine galactosidase. Biochem. Biophys. Res. Commun. 48: 538, 1972.
Miyatake, T. and Suzuki, K.: Galactosyl sphingosine galactosyl hydrolase in rat brain: Probable identity with galactosylceramide galactosyl hydrolase. J. Neurochem. 22: 231, 1974.
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.
Schutta, H.S. and Neville, H.E.: Effects of cholesterol biosynthesis inhibitors on the nervous system. Lab. Invest. 19: 487, 1968.
Suzuki, K.: Ultrastructural study of experimental globoid cells. Lab. Invest. 23: 612, 1970.
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.
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.
Tanaka, H. and Suzuki, K.: Lactosylceramide β-galactosidase in human sphingolipidoses: Evidence for two genetically distinct enzymes. J. Biol. Chem. 250: 2324, 1975.
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.
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.
Wenger, D.A.: Studies on galactosylceramide and lactosylceramide β-galactosidase. Chem. Phys. Lipids 13: 327, 1974.
Wenger, D.A., Sattler, M. and Hiatt, W.: Globoid cell leukodystrophy: Deficiency of lactosyl ceramide beta-galactosidase. Proc. Nat. Acad. Sci. 71: 854, 1974.
Yunis, E.J. and Lee, R.E.: The ultrastructure of globoid (Krabbe) leukodystrophy. Lab. Invest. 21: 415, 1969.
Yunis, E. and Lee, R.E.: Further observations on the fine structure of globoid leukodystrophy. Human Pathology 3: 371, 1972.
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 1976 Plenum Press, New York
About this chapter
Cite this chapter
Suzuki, K., Tanaka, H., Suzuki, K. (1976). Studies on the Pathogenesis of Krabbe’s Leukodystrophy: Cellular Reaction of the Brain to Exogenous Galactosylsphingosine, Monogalactosyl Diglyceride, and Lactosylceramide. In: Volk, B.W., Schneck, L. (eds) Current Trends in Sphingolipidoses and Allied Disorders. Advances in Experimental Medicine and Biology, vol 68. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-7735-1_7
Download citation
DOI: https://doi.org/10.1007/978-1-4684-7735-1_7
Publisher Name: Springer, Boston, MA
Print ISBN: 978-1-4684-7737-5
Online ISBN: 978-1-4684-7735-1
eBook Packages: Springer Book Archive