Suppressed UDP-galactose: Ceramide galactosyltransferase and myelin protein mRNA in twitcher mouse brain

Masako Taniike, Jill R. Marcus, Toshinori Nishigaki, Nobuya Fujita, Brian Popko, Kunihiko Suzuki, Kinuko Suzuki

Research output: Contribution to journalArticlepeer-review

20 Scopus citations


The developmental changes in expression of steady-state mRNA that encode proteins that are important for myelination (myelin basic protein, myelin- associated glycoprotein, proteolipid protein, UDP-galactose: ceramide galactosyltransferase) and glial fibrillary acidic protein were investigated in the brain of the twitcher mouse, a model of human globoid cell leukodystrophy. This disease is caused by a mutation in the gene encoding the lysosomal enzyme, galactosylceramidase, which catalyzes the degradation of the myelin lipid galactosylceramide. Before postnatal day (PND) 20, the levels of myelin protein mRNA were similar in twitcher and normal mice. With progression of demyelination after PND 25-30, myelin protein mRNA levels gradually decreased. The period of maximum expression of the myelin protein genes in twitcher mice was, however, similar to that of normal control mice. mRNA levels for the gene that encodes the enzyme UDP-galactose:ceramide galactosyitransferase which is responsible for catalyzing the final step in galactosyiceramide synthesis, was exceptionally downregulated from the early stages of the disease. The increase of glial fibrillary acidic protein (GFAP) mRNA levels preceded morphological evidence of demyelination.

Original languageEnglish (US)
Pages (from-to)536-540
Number of pages5
JournalJournal of Neuroscience Research
Issue number4
StatePublished - Feb 15 1998


  • Demyelination
  • Glial fibrillary acidic protein
  • Psychosine
  • Twitcher mouse
  • UDP-galactose:eramide galactosyltransferase

ASJC Scopus subject areas

  • Cellular and Molecular Neuroscience


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