Gerstmann-Sträussler-Scheinker: a new phenotype with 'curly' PrP deposits.

  title={Gerstmann-Str{\"a}ussler-Scheinker: a new phenotype with 'curly' PrP deposits.},
  author={Monica Colucci and Francisco J. Moleres and Zhi-Liang Xie and Abhik Ray-Chaudhury and Sujata Gutti and Cathrin M Butefisch and Larisa Cerven{\'a}kov{\'a} and Wen Wang and Lev G. Goldfarb and Qingzhong Kong and Bernardino Ghetti and Shu Guang Chen and Pierluigi Gambetti},
  journal={Journal of neuropathology and experimental neurology},
  volume={65 7},
Gerstmann-Sträussler-Scheinker (GSS) is a hereditary prion disease typically associated with prion protein (PrP)-containing plaques. The protease-resistant, scrapie PrP (PrPSc) is represented by internal fragments, whereas the C-terminal fragments associated with the other prion diseases are generally underrepresented. Different histopathologic and PrPSc features associated with at least 13 PrP gene (PRNP) mutations have been described in GSS. We report the histopathology and PrP… CONTINUE READING
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