EAAT4 mRNA expression is preserved in the cerebellum of prion protein-deficient mice

To study the mechanism underlying the selective degeneration of Purkinje cells in the cerebellum of the Nagasaki (Ngsk) prion protein-deficient (PrP(-/-)) mice, the mRNA levels of glutamate transporter EAAT4, the marker highly specific for Purkinje cell synapses, were analyzed by semi-quantitative reverse transcription-polymerase chain reaction. EAAT4 mRNA was expressed in the cerebellum of PrP(-/-) mice presenting with cerebellar ataxia, at the levels identical to those in the cerebellum of non-ataxic PrP(+/-) mice. Furthermore, EAAT4 mRNA was identified in the cerebrum of both PrP(-/-) and PrP(+/-) mice, although its levels were much lower than those in the cerebellum. These results indicate that Purkinje cell degeneration found in the cerebellum of PrP(-/-) mice is not primarily caused by glutamate neurotoxicity, although it remains to be investigated whether preserved expression of EAAT4 might represent a compensatory mechanism for protecting against Purkinje cell degeneration in the PrP(-/-) mice cerebellum.