National Institute of Animal Health (NIAH)

Topics in Animal Health Research 2013

12. Conformation specific epitopes located in the N-terminal region of abnormal prion protein (PrPSc)

Japanese

Prion diseases are fatal neurological disease which cause spongiform changes in the brain associated with an accumulation of abnormal isoform of prion protein (PrPSc). PrPSc is a disease isoform of the host-encoded cellular prion protein (PrPC). The conformation of PrPSc differs from that of PrPC, but the precise characteristics of PrPSc remain to be elucidated. To clarify the properties of PrPSc, we attempted to generate PrPSc-specific monoclonal antibodies (mAbs). The generated mAbs, 8D5 and 6A12, selectively reacted with PrPSc from the brains of prion-affected mice, sheep, and cattle, but did not react with PrPC from the brains of healthy animals. Epitope analysis revealed that mAbs 8D5 and 6A12 recognized the PrP subregion at amino acid positions 31–39 and 41–47, respectively. This indicates that conformation-specific epitopes exist in the N-terminal region of PrPSc, and that mAbs 8D5 and 6A12 are powerful tools to detect authentic PrPSc.
(Influenza and Prion Disease Research Center)

References:

Masujin K. et al (2013) PLoS One 8(2):e58013

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