ArticleActa neuropathologica1984
Demyelination in the spinal cord of murine globoid cell leukodystrophy (the twitcher mouse).
Article in Acta neuropathologica, 1984. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 38 citations in OpenAlex.
- Article
- Deficiency of galactosyl-ceramidase in adult oligodendrocytes worsens disease severity during chronic experimental allergic encephalomyelitis.Molecular therapy : the journal of the American Society of Gene Therapy · 2024Article
- Krabbe disease: New hope for an old disease.Neuroscience letters · 2021Review
- Krabbe Disease: Prospects of Finding a Cure Using AAV Gene Therapy.Frontiers in medicine · 2021Review
- Lipids in the Physiopathology of Hereditary Spastic Paraplegias.Frontiers in neuroscience · 2020Review
- AAV-Mediated GALC Gene Therapy Rescues Alpha-Synucleinopathy in the Spinal Cord of a Leukodystrophic Lysosomal Storage Disease Mouse Model.Frontiers in cellular neuroscience · 2020Article
- NovelFrontiers in neurology · 2020Article
- Psychosine enhances the shedding of membrane microvesicles: Implications in demyelination in Krabbe's disease.PloS one · 2017Article
- Generation of a LacZ reporter transgenic mouse line for the stereological analysis of oligodendrocyte loss in galactosylceramidase deficiency.Journal of neuroscience research · 2016Article
- Mechanism of neuromuscular dysfunction in Krabbe disease.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2015Article
- Missense mutation in mouse GALC mimics human gene defect and offers new insights into Krabbe disease.Human molecular genetics · 2013Article
- MMP-3 mediates psychosine-induced globoid cell formation: implications for leukodystrophy pathology.Glia · 2013Article
- Peripheral neuropathy in the Twitcher mouse involves the activation of axonal caspase 3.ASN neuro · 2011Review
- 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 · 2009Article
- Accumulation of galactosylsphingosine (psychosine) does not interfere with phosphorylation and methylation of myelin basic protein in the twitcher mouse.Neurochemical research · 1990Article
- Metabolism of exogenous galactosylceramide in the twitcher mouse brain.Neurochemical research · 1989Article
- Expression of glial fibrillary acidic protein in the CNS and PNS of murine globoid cell leukodystrophy, the twitcher.The American journal of pathology · 1986Article
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
Abstract
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.
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