Macrophages Counteract Demyelination in a Mouse Model of Globoid Cell Leukodystrophy
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چکیده
منابع مشابه
Macrophages counteract demyelination in a mouse model of globoid cell leukodystrophy.
Whether microglia and macrophages are beneficial or harmful in many neurological disorders, including demyelinating diseases such as multiple sclerosis and the leukodystrophies, is currently under debate. Answering this question is of special interest in globoid cell leukodystrophy (GLD), a genetic fatal demyelinating disease, because its rapidly progressive demyelination in the nervous system ...
متن کامل[Globoid cell leukodystrophy].
A 29 year old male with onset of globoid cell leukodystrophy at age 14 is described. This is the first case of enzymatically confirmed globoid cell leukodystrophy with onset of symptoms after the age of ten. This patient is unique because of the late onset and slow progression and extends the clinical spectrum of globoid cell leukodystrophy. Globoid cell leukodystrophy is caused by the deficien...
متن کاملHematopoietic stem-cell transplantation in globoid-cell leukodystrophy.
BACKGROUND Globoid-cell leukodystrophy is caused by a deficiency of galactocerebrosidase, which results in progressive central nervous system deterioration. We investigated whether allogeneic hematopoietic stem-cell transplantation can provide a source of leukocyte galactocerebrosidase and thereby prevent the decline of central nervous system function in patients with the disease. METHODS Fiv...
متن کاملGloboid cell-like leukodystrophy in a domestic longhaired cat.
Globoid cell leukodystrophy (GLD; Krabbe disease), is a rare heritable metabolic disorder in humans, dogs, mutant twitcher mice, and rhesus monkeys that is caused by a deficiency in the lysosomal enzyme galactocerebrosidase (GALC). GALC deficiency results in the accumulation of psychosine, which is toxic to oligodendrocytes and Schwann cells of the central and peripheral nervous systems. Clinic...
متن کاملgloboid cell leukodystrophy (krabbe disease)
how to cite this article: tavasoli a. globoid cell leukodystrophy (krabbe disease). iran j child neurol. autumn 2014;8;4(suppl.1):14-15. pls see pdf.
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ژورنال
عنوان ژورنال: Journal of Neuroscience
سال: 2011
ISSN: 0270-6474,1529-2401
DOI: 10.1523/jneurosci.6344-10.2011