L-Glutamate toxicity in Huntington's disease fibroblasts |
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Authors: | P N Gray P C May L Mundy J Elkins |
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Affiliation: | Department of Biochemistry and Molecular Biology, University of Oklahoma at Oklahoma City, Health Sciences Center, Oklahoma City, OK 73190 USA |
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Abstract: | Brain degeneration in Huntington's Disease is thought to occur primarily in the regions of high L-glutamate concentrations. Huntington's Disease fibroblast cultures have a sensitivity to these high concentrations of L-glutamate. These cells show degeneration and loss of viability, within 12 hrs, following treatment with 30 mM L-glutamate. This effect appears to be specific for L-glutamate, can be prevented by glutamine, and is not observed in matched control cultures. The observed glutamate sensitivity may serve as a biochemical genetic marker and the excessive effect of glutamate on neuronal membranes could initiate the characteristic neuronal degeneration observed in Huntington's Disease brains. |
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Keywords: | HD Huntington's Disease |
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