Lurcher mutant mice are characterized by massive degeneration of the c
erebellum, including Purkinje cells and granule cells, as well as for
the loss of neurons from the inferior olive, Concentrations of dopamin
e and two of its metabolites and of several amino acid neurotransmitte
rs were determined in the cerebellum and in other brain regions of the
se mutants. By comparison to wild-type mice of the same background str
ain, glutamate and taurine concentrations were reduced in the Lurcher
cerebellum, No decrease was found for aspartate, gamma-aminobutyric ac
id (GABA), glycine, as well as dopamine and its metabolites. Moreover,
no neurochemical alterations occurred in the brain stem, thalamus, or
neostriatum of Lurcher mutants, A selective reduction of glutamate co
ncentration was found in the hippocampus, while all amino acids measur
ed were decreased in the entorhinal-piriform areas. These results indi
cate region-selective reductions of neurotransmitter concentrations in
a mouse mutant with a defined cerebellar cortical pathology. (C) 1998
Elsevier Science Inc.