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Article

Establishment and characterization of spontaneously immortalized Schwann cells from murine model of globoid cell leukodystrophy (twitcher).

Journal of neuroscience research - 1 Jun 2002

Shen Jin-Song, Watabe Kazuhiko, Meng Xing-Li, Ida Hiroyuki, Ohashi Toya, Eto Yoshikatsu

Abstract excerpt

The twitcher mouse is a murine model of human globoid cell leukodystrophy (GLD; Krabbe disease) caused by a genetic defect in the activity of galactosylceramidase (GALC). An accumulation of cytotoxic metabolite, galactosylsphingosine (psychosine), in myelin forming cells (oligodendrocytes and Schwann cells) of the twitcher mouse as well as patients with GLD has been suggested to cause dysfunction of these cells...

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Source

Identifiers

PMID: 12111848
DOI: 10.1002/jnr.10247

Genes

GALC

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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