Abstract |
The twitcher mouse is a murine model of globoid cell leukodystropy, a genetic demyelinating disease caused by a mutation of the galactosylceramidase gene. Demyelination of the central nervous system commences around 20 postnatal days. Using GFP-transgenic mice as donors, the distribution of hematogenous cells after bone marrow transplantation was investigated in the twitcher mice. Bone marrow transplantation was carried out at 8 postnatal days. In twitcher chimeric mice examined before 30 postnatal days, numerous GFP(+) cells were detected in spleen and peripheral nerve but only a few were detected in the liver, lung, and spinal white matter. In contrast, at 35 to 40 postnatal days when demyelination is evident, many GFP(+) cells with ameboid form were detected in the white matter of the spinal cord, brainstem, and cerebrum. Approximately half of these GFP(+) cells were co-labeled with Mac-1. In twitcher chimeric mice examined after 100 postnatal days, the majority of GFP/Mac-1 double-positive cells displayed the morphological features of ramified microglia with fine delicate processes and was distributed diffusely in both gray and white matter. These results suggest that a significant number of donor hematogenous cells are able to infiltrate into the brain parenchyma, repositioning themselves into areas previously occupied by microglia, and to ameliorate lethality.
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Authors | Y P Wu, E McMahon, M R Kraine, R Tisch, A Meyers, J Frelinger, G K Matsushima, K Suzuki |
Journal | The American journal of pathology
(Am J Pathol)
Vol. 156
Issue 6
Pg. 1849-54
(Jun 2000)
ISSN: 0002-9440 [Print] United States |
PMID | 10854208
(Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S.)
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Chemical References |
- Indicators and Reagents
- Luminescent Proteins
- Green Fluorescent Proteins
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Topics |
- Animals
- Blood Cells
(metabolism, transplantation)
- Bone Marrow Transplantation
- Central Nervous System
(metabolism)
- Green Fluorescent Proteins
- Indicators and Reagents
- Luminescent Proteins
(blood, genetics, metabolism)
- Mice
- Mice, Inbred C57BL
- Mice, Neurologic Mutants
- Mice, Transgenic
- Peripheral Nerves
(metabolism)
- Postoperative Care
- Reference Values
- Tissue Distribution
- Tissue Donors
- Viscera
(metabolism)
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