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Cellular prion protein changes conformation during transformation to an infectious scrapie isoform. One measure of transformation is the development of partial resistance to protease treatment. A fraction of human and bovine plasma was identified containing activity that facilitates transformation of cellular prion protein to a protease resistant isoform in the presence of RNA in the absence of seeded scrapie prion protein. Purification of proteins from this fraction led to the identification of alpha2-macroglobulin as an active component suggesting that it may facilitate conformational changes in prion protein in spontaneous forms of prion disease.
Adler et al. (Mon,) studied this question.
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