Pathology of the peripheral nerve in the twitcher mouse following bone marrow transplantation

Akira Kondo, Peter M. Hoogerbrugge, Kinuko Suzuki, Ben J.H.M. Poorthuis, Dirk W. Van Bekkum, Kunihiko Suzuki

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3 Citations (Scopus)


The peripheral nerve of the homozygous twitcher mouse (twi/twi), a murine model of globoid cell leukodystrophy (GLD), was examined following bone marrow transplantation (BMT). The light and electron microscopic studies revealed markedly increased numbers of remyelinated fibers and almost complete disappearance of the typical inclusion-laden macrophages in the trigeminal and sciatic nerves of the twi/twi which survived beyond 100 days of age. The pattern of remyelination appeared to be normal. GLD inclusions were still observed in the cytoplasm of some of the remyelinating Schwann cells and demyelinated fibers were still present in 108-day-old twitcher although no features of active demyelinating processes were observed. Thus, basic metabolic abnormality is still present despite clinical improvement in the twi/twi mouse following BMT.

Original languageEnglish
Pages (from-to)178-183
Number of pages6
JournalBrain Research
Issue number1
Publication statusPublished - 13 Sept 1988
Externally publishedYes


  • Bone marrow transplantation
  • Globoid cell leukodystrophy
  • Peripheral nerve
  • Remyelination
  • Twitcher mouse


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