Glutamate in Creutzfeldt-Jakob Disease CJD

CJD is the most prevalent form of prion disease in humans. CJD is characterized by neuronal loss, astrocytic gliosis, and accumulation of an abnormal extracellular /3-helix rich prion protein (PrPsc). This abnormal protein is an isoform of a normally 

The antimalarial drug quinacrine and some phenothiazine derivatives, acepro-mazine, chlorpromazine, and promazine, have been used for the treatment of prion diseases (Doh-ura et al., 2000 Korth et al., 2001 May et al., 2003). The molecular mechanism associated with the inhibition of PrPsc formation by quinacrine remains unknown. However, it is proposed that quinacrine binds with human prion protein at the Tyr-225, Tyr-226, and Gln-227 residues of helix 3 (Vogtherr et al., 2003) and provides neuroprotection. Quinacrine may also act as an antioxidant and reduce the toxicity of prP 6 (Turnbull et al., 2003). 

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