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C B Fluharty, J Johnson, J Whitelegge, K F Faull, A L Fluharty
Cerebroside sulfate activator (saposin B) is a small protein involved in glycosphingolipid metabolism. It binds certain membrane lipids, making them available to water-soluble enzymes. Defects in this protein are responsible for a form of metachromatic leukodystropy, a progressive neurodegenerative condition. The protein participates in the catabolism of a number of lipids but does show lipid binding selectivity. However, the basis of this selectivity is unclear. Here we assess the relative binding of a number of lipids compared to cerebroside sulfate (sulfatide)...
January 1, 2001: Journal of Neuroscience Research
Y P Wu, E McMahon, M R Kraine, R Tisch, A Meyers, J Frelinger, G K Matsushima, K Suzuki
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...
June 2000: American Journal of Pathology
E Costantino-Ceccarini, A Luddi, M Volterrani, M Strazza, M A Rafi, D A Wenger
Krabbe disease or globoid cell leukodystropy is a lysosomal disorder caused by a deficiency of galactocerebrosidase (GALC) activity. This results in defects in myelin that lead to severe symptoms and early death in most human patients and animals with this disease. With the cloning of the GALC gene and the availability of the mouse model, called twitcher, it was important to evaluate the effects of providing GALC via a retroviral vector to oligodendrocytes in culture. After differentiation, the untransduced cells from normal mice extended highly branched processes while those from the twitcher mice did not...
February 1999: Neurochemical Research
L Pandit, R Kapadia, P Kini, S Rao
No abstract text is available yet for this article.
June 1994: Indian Pediatrics
H Tanaka, M Arima, Y Suzuki, K Takahashi
No abstract text is available yet for this article.
June 1975: Rinshō Shinkeigaku, Clinical Neurology
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