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Fig. 3 | Acta Neuropathologica Communications

Fig. 3

From: Gerstmann-Sträussler-Scheinker disease revisited: accumulation of covalently-linked multimers of internal prion protein fragments

Fig. 3

Relative abundance of 129M and 129V PrP variants in resPrPD associated with GSSA117V and GSSF198S. a: GSSA117V; b: GSSF198S. The relative abundance of resPrPD with M or V at residue 129 reflects the representation of the PrP mutation, which is coupled with the 129V in both GSS variants. Approximately 10–25% of resPrPD could be identified as non-mutated (wild type) in GSSF198S while only mutated resPrPD could be detected in GSSA117V. The relative populations were determined by mass spectrometry using the spectral counting method

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