ArticleActa neuropathologica1994
Spacio-temporal progression of demyelination in twitcher mouse: with clinico-pathological correlation.
Article in Acta neuropathologica, 1994. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers.
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Who cites it
32 citing papers in PubMed, 61 citations in OpenAlex.
- Article
- Neuroimmunology in globoid cell leukodystrophy: A comprehensive review including treatments, models, and neuroimmune mechanisms underlying neuropathology.Journal of neuroimmunology · 2025Review
- Ablation of lipocalin-2 reduces neuroinflammation in a mouse model of Krabbe disease.Scientific reports · 2024Article
- Article
- Upregulation of non-canonical and canonical inflammasome genes associates with pathological features in Krabbe disease and related disorders.Human molecular genetics · 2023Article
- Neuron-specific ablation of the Krabbe disease gene galactosylceramidase in mice results in neurodegeneration.PLoS biology · 2022Article
- Efficacy and Safety of a Krabbe Disease Gene Therapy.Human gene therapy · 2022Article
- CRISPR-Cas9 Knock-In of T513M and G41S Mutations in the Murine β-Galactosyl-Ceramidase Gene Re-capitulates Early-Onset and Adult-Onset Forms of Krabbe Disease.Frontiers in molecular neuroscience · 2022Article
- Expression of Ripk1 and DAM genes correlates with severity and progression of Krabbe disease.Human molecular genetics · 2021Article
- Review
- Article
- A HILIC-MS/MS method for simultaneous quantification of the lysosomal disease markers galactosylsphingosine and glucosylsphingosine in mouse serum.Biomedical chromatography : BMC · 2018Article
- Psychosine enhances the shedding of membrane microvesicles: Implications in demyelination in Krabbe's disease.PloS one · 2017Article
- Metabolic profiling reveals biochemical pathways and potential biomarkers associated with the pathogenesis of Krabbe disease.Journal of neuroscience research · 2016Article
- Treatment for Krabbe's disease: Finding the combination.Journal of neuroscience research · 2016Review
- Inherited and acquired disorders of myelin: The underlying myelin pathology.Experimental neurology · 2016Review
- 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
- Psychosine induces the dephosphorylation of neurofilaments by deregulation of PP1 and PP2A phosphatases.Neurobiology of disease · 2012Article
- Effect of intrastriatal mesenchymal stromal cell injection on progression of a murine model of Krabbe disease.Behavioural brain research · 2011Article
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
Abstract
The twitcher (twi/twi) is an authentic murine model of human globoid cell leukodystrophy (GLD), caused by a deficiency of galactosylceramidase. Similar to human GLD, the twitcher shows progressive deterioration of neurological function and its neuropathology is characterized by a collection of periodic acid-Schift stain (PAS)-positive macrophages in the areas of demyelination. However, there are some differences in the clinico-pathological aspects between human and murine GLD. We investigated the spacio-temporal progression of neuropathology in the twitcher from postnatal day (PND) 10 to 45. No clinical symptoms or neuropathological changes were apparent in twi/twi until PND 15. Generally, infiltration of macrophages, concomitant with myelin degeneration, was recognized in the cerebellar white matter and the brain stem after PND 20, then in cerebral white matter after PND 25, and in cerebral and cerebellar gray matter after PND 30. The demyelination was very severe in the radix of the 8th and the 5th cranial nerves. The neurological symptoms such as tremor, spasticity and cranial nerve dysfunction were well correlated with the progression of pathological changes. Demyelination progressed in an orderly fashion such that myelin degeneration began 10 to 20 days after the commencement of myelination in any of the given nerve fiber tracts. This suggests that there are no significant differences in the metabolism of galactocerebroside in the myelin and myelin-forming cells in individual nerve fiber tracts throughout the murine brain. Over-expression of glial fibrillary acidic protein was already present before the initiation of obvious demyelination.(ABSTRACT TRUNCATED AT 250 WORDS)
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