Abstract
The toxicity of l-glutamic acid toward cultured fibroblasts and lymphocytes from age-matched, sex-matched controls, patients with Huntington's disease (HD) and patients with other diseases has been studied. In an initial non-blinded study, l-glutamic acid (15 mM) exerted a significant toxic effect on HD fibroblasts, decreasing viability by approximately 60% after 48 h exposure. The magnitude of the toxic effect on HD fibroblasts as a group was significantly different from the mean effect on the normal control group ( P < 0.003) and non-HD control group ( P < 0.004). However, there was variability in the sensitivity of a given fibroblast culture to l-glutamate. This toxic effect was also seen in several normal control and non-HD control fibroblasts. In a second blinded study using cultured fibroblasts from HD patients and age-matched, sexmatched controls, we were unable to distinguish between HD and control cultures. No difference in l-glutamate toxicity was observed between control and HD lymphocytes in short-term cultures. We conclude that the toxicity of l-glutamate is not specific for HD cells and that this experimental approach will be of little value in identifying cells from patients with HD.
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