Transmissible spongiform encephalopathy (TSE) is a neurodegenerative disease characterized by spongiform degeneration and accumulation of an infectious isoform (PrP Sc ) of the prion protein in the central nervous system. PrP Sc originates from a ubiquitous cellular prion protein (PrP C ). We attempted to develop an easy method of quantitative analysis of PrP by immunoblotting based on densitometry data for PrP bands in immunoblots. Both PrP C and PrP Sc yield three bands in immunoblots, and

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... y correspond to PrP molecules carrying two, one, and no Asn-linked sugar chains. We used bovine PrP C as a model protein in the immunoblotting study. We removed the Asn-linked sugar chains from the PrP molecules with N-glycanase to convert all three glycoforms of PrP into a single band of the deglycosylated form and determined the PrP by densitometry calibrated with recombinant bovine PrP.