Two transgenic strains of mutant mice lacking hypoxanthine-guanidine
phosphoribosyltransferase (HPRT) activity were examined behaviorally and
neurochemically for phenotypic similarity to the human Lesch-Nyhan syndrome. In this
syndrome, male children markedly deficient in the enzyme HPRT develop self-mutilation
and severe motoric difficulties, and exhibit a pronounced deficiency of dopamine in the
basal ganglia. The HPRT-deficient mice showed no evidence of self-mutilation, no …