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Publication : Impaired glutamate uptake in the R6 Huntington's disease transgenic mice.

First Author  Liévens JC Year  2001
Journal  Neurobiol Dis Volume  8
Issue  5 Pages  807-21
PubMed ID  11592850 Mgi Jnum  J:72696
Mgi Id  MGI:2153409 Doi  10.1006/nbdi.2001.0430
Citation  Lievens JC, et al. (2001) Impaired glutamate uptake in the r6 huntington's disease transgenic mice. Neurobiol Dis 8(5):807-21
abstractText  Huntington's disease (HD) is a late-onset neurodegenerative disease for which the mutation is CAG/polyglutamine repeat expansion. The R6 mouse lines expressing the HD mutation develop a movement disorder that is preceded by the formation of neuronal polyglutamine aggregates. The phenotype is likely caused by a widespread neuronal dysfunction, whereas neuronal cell death occurs late and is very selective. We show that a decreased mRNA level of the major astroglial glutamate transporter (GLT1) in the striatum and cortex of these mice is accompanied by a concomitant decrease in glutamate uptake. In contrast, the expression of the glutamate transporters, GLAST and EAAC1, remain unchanged. The mRNA level of the astroglial enzyme glutamine synthetase is also decreased. These changes in expression occur prior to any evidence of neurodegeneration and suggest that a defect in astrocytic glutamate uptake may contribute to the phenotype and neuronal cell death in HD.
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