In the central nervous system and particularly in the striatum of patients with Huntington's disease (HD) a dramatic cell loss can be observed. Animal models of HD are based on intrastriatal injection of excitatory amino acids (EAAs). Stimulation of EAA receptors for a prolonged period of time degenerates the cells on which the EAA receptors are located, a phenomenon known as excitotoxicity. Several categories of EAA receptors, viz. quisqualate, kainate and N-methyl-D-aspartate (NMDA), have been identified in the central nervous system. Interestingly, quinolinic acid, a metabolite of tryptophan along the kynurenine pathway, appeared to be an agonist on the NMDA receptor and a potent excitotoxin. Indications have been reported, although still controversial, for derangements in the formation of quinolinic acid to occur in the brains of patients with HD. Based on these studies the likeliness of a role for quinolinic acid in the etiology of HD is evaluated.