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Galactosylsphingosine in Murine and Human Tissues of Normal and Globoid Cell Leukodystrophy Cases

  • Takuro Kobayashi
  • Hisaharu Shinoda
  • Ikuo Goto
Conference paper
Part of the NATO ASI Series book series (NSSA, volume 150)

Abstract

Globoid cell leukodystrophy (Krabbe’s disease, GLD) is a neurological dijogder of infancy caused by a genetic deficiency of galactosyl-ceramidase I.1,2 The murine analogue is twitcher mouse, in which galactosyl-ceramidase I is also deficient.3,4 The pathological features are similar to those of human GLD. In both, severe demyelination occurs in central and peripheral nervous tissues. In addition, gliosis, loss of oligodendrocytes, and presence of globoid cells are evident in the white matter. Galactosylceramide, a natural substrate of the deficient enzyme, apparently does not accumulate in the nervous tissues of human and murine cases of GLD. In 1972, Miyatake and Suzuki6 found that the hydrolysis of galactosylsphingosine (psychosine) is impaired in tissues from GLD patients and proposed, for the pathogenesis of GLD, the “psychosine hypothesis” which postulates that the degeneration of oligodendrocytes and consequent demyelination in the GLD tissue are due to the toxicity of galactosylsphingosine. In 1980, Svennerholm et al demonstrated an abnormal accumulation of galactosylsphingosine in the brains of GLD patients and supported the hypothesis. As to the twitcher mouse, Igisu and Suzuki8 noted an accumulation of the compound, determined by fluorometric assay using TLC. In this paper, we describe a procedure for the determination of galactosylsphingosine, using HPLC, and data on galactosylsphingosine content in tissues from normal and twitcher mice. The data on normal human tissues and tissues from a patient with GLD are also described.

Keywords

Spinal Cord Sciatic Nerve Nervous Tissue Normal Human Tissue Twitcher Mouse 
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.

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Copyright information

© Plenum Press, New York 1988

Authors and Affiliations

  • Takuro Kobayashi
    • 1
  • Hisaharu Shinoda
    • 1
  • Ikuo Goto
    • 1
  1. 1.Department of Neurology, Neurological Institute Faculty of MedicineKyushu University 60Fukuoka 812Japan

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