Altered properties of amyloidogenic prion protein in genetic...

Altered properties of amyloidogenic prion protein in genetic Creutzfeldt–Jakob disease with PRNP V180I mutation in response to pentosan polysulfate
https://onlinelibrary.wiley.com/doi/full/10.1111/bpa.13197

"Both PPS-treated cases showed long survival time over 5 years from onset and increased PrP deposition with a characteristic pattern of coarse granular depositions and congophilic PrP microspheres, whereas the cases without PPS showed around 1-year survival from onset and relatively mild neuronal loss and synaptic PrP deposition."

"These unique biochemical and histopathological changes may reflect the progression of V180I gCJD and its modification by PPS, suggesting the possible existence of toxic PrP-oligomer in the pathophysiology of V180I gCJD and beneficial effects of PPS toward the aggregation and detoxication of toxic PrP-oligomer."