EurekaMag.com logo
+ Site Statistics
References:
53,623,987
Abstracts:
29,492,080
+ Search Articles
+ Subscribe to Site Feeds
EurekaMag Most Shared ContentMost Shared
EurekaMag PDF Full Text ContentPDF Full Text
+ PDF Full Text
Request PDF Full TextRequest PDF Full Text
+ Follow Us
Follow on FacebookFollow on Facebook
Follow on TwitterFollow on Twitter
Follow on LinkedInFollow on LinkedIn

+ Translate

Demyelination in the spinal cord of murine globoid cell leukodystrophy (the twitcher mouse)



Demyelination in the spinal cord of murine globoid cell leukodystrophy (the twitcher mouse)



Acta Neuropathologica 62(4): 298-308



Chronologic events of demyelination were investigated in the spinal cord of the twitcher mouse, an authentic murine model of human globoid cell leukodystrophy (GLD) from 5 to 45 days postnatal. There was very little evidence of myelin degeneration before day 25 although clustered or scattered globoid cells were already noted in the dorsal columns and intramedullary portion of the ventral roots. Globoid cells contained typical cytoplasmic inclusions and in those which were found adjacent to degenerating myelin and naked axons, myelin debris were conspicuous in their cytoplasm. Vesiculation of myelin and a feature of globoid cells stripping myelin lamellae were noted in the area of demyelination. Myelin and oligodendroglial degeneration became pronounced throughout the spinal white matter after day 40 but globoid cells tended to be more concentrated in the dorsal columns. Our observations suggest that the emergence of globoid cells in GLD is in response to the changes in biochemical environment (i.e., excessive presence of galactosylceramide in the tissue?), and these cells appear to have a role as phagocytic cells in removing myelin lamellae.

(PDF emailed within 0-6 h: $19.90)

Accession: 042751905

Download citation: RISBibTeXText

PMID: 6730907

DOI: 10.1007/bf00687612



Related references

Diffusion tensor imaging detects axonal injury and demyelination in the spinal cord and cranial nerves of a murine model of globoid cell leukodystrophy. Nmr in Biomedicine 22(10): 1100-1106, 2010

Murine globoid cell leukodystrophy: the twitcher mouse. An ultrastructural study of the kidney. Laboratory Investigation; A Journal of Technical Methods and Pathology 50(1): 42-50, 1984

Nerve conduction studies in the Twitcher mouse (murine globoid cell leukodystrophy). Journal of the Neurological Sciences 74(2-3): 307-318, 1986

Murine globoid cell leukodystrophy (the twitcher mouse). The presence of characteristic inclusions in the kidney and lymph nodes. American Journal of Pathology 112(2): 147-154, 1983

Hematopoietic cell transplantation after administration of high-dose busulfan in murine globoid cell leukodystrophy (the twitcher mouse). Pediatric Research 29(3): 302-305, 1991

The effects of demyelinating neuropathy on the soleus muscle in hematopoietic cell transplantation of the twitcher mouse murine globoid cell leukodystrophy. Neurology 36(4 SUPPL 1): 301, 1986

Hematopoietic cell transplantation in murine globoid cell leukodystrophy (the twitcher mouse): effects on levels of galactosylceramidase, psychosine, and galactocerebrosides. Proceedings of the National Academy of Sciences of the United States of America 84(12): 4259-4263, 1987

Hematopoietic cell transplantation hct in murine globoid cell leukodystrophy gcl the twitcher mouse a model of human sphingolipid storage krabbe disease. Experimental Hematology (Charlottesville) 15(5): 449, 1987

Biochemical and morphological studies of dorsal root ganglion and its cultured cells from twitcher mouse (murine globoid cell leukodystrophy). Brain & Development 12(4): 412-416, 1990

Globoid cell leukodystrophy: specialized contact of globoid cell with astrocyte in the brain of twitcher mouse. Acta Neuropathologica 58(4): 237-242, 1982

Severe graft versus host disease in a patient with globoid cell leukodystrophy following umbilical cord blood transplantation: resemblance to the twitcher mouse model. Turkish Journal of Pediatrics 49(3): 304-306, 2007

Immunohistochemistry of the central nervous system in murine globoid cell leukodystrophy the twitcher. Journal of Neurochemistry 44(SUPPL): S174, 1985

An apoptotic depletion of oligodendrocytes in the twitcher, a murine model of globoid cell leukodystrophy. Journal of Neuropathology and Experimental Neurology 58(6): 644-653, 1999

The twitcher mouse A model of human globoid cell leukodystrophy. Martenson, R E Myelin: Biology and chemistry ix+958p CRC Press, Inc : Boca Raton, Florida, USA; London, England, UK Illus ISBN 0-8493-8849-X : 745-759, 1992

Expression of glial fibrillary acidic protein in the CNS and PNS of murine globoid cell leukodystrophy, the twitcher. American Journal of Pathology 125(2): 227-243, 1986