Intracellular mechanisms mediating the neuronal death and astrogliosis induced by the prion protein fragment 106-126

Stefano Thellung, Tullio Florio, Alessandro Corsaro, Sara Arena, Massimo Merlino, Mario Salmona, Fabrizio Tagliavini, Orso Bugiani, Gianluigi Forloni, Gennaro Schettini

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Prion encephalopathies include fatal diseases of the central nervous system of men and animals characterized by nerve cell loss, glial proliferation and deposition of amyloid fibrils into the brain. During these diseases a cellular glycoprotein (the prion protein, PrP(C)) is converted, through a not yet completely clear mechanism, in an altered isoform (the prion scrapie, PrP(Sc)) that accumulates within the brain tissue by virtue of its resistance to the intracellular catabolism. PrP(Sc) is believed to be responsible for the neuronal loss that is observed in the prion disease. The PrP 106-126, a synthetic peptide that has been obtained from the amyloidogenic portion of the prion protein, represents a suitable model for studying the pathogenic role of the PrP(Sc), retaining, in vitro, some characteristics of the entire protein, such as the capability to aggregate in fibrils, and the neurotoxicity. In this work we present the results we have recently obtained regarding the action of the PrP 106-126 in different cellular models. We report that the PrP 106-126 induces proliferation of cortical astrocytes, as well as degeneration of primary cultures of cortical neurons or of neuroectodermal stable cell lines (GH3 cells). In particular, these two opposite effects are mediated by the same attitude of the peptide to interact with the L-type calcium channels: in the astrocytes, the activity of these channels seems to be activated by PrP 106-126, while, in the cortical neurons and in the GH3 cells, the same treatment causes a blockade of these channels causing a toxic effect. Copyright (C) 2000 ISDN.

Original languageEnglish
Pages (from-to)481-492
Number of pages12
JournalInternational Journal of Developmental Neuroscience
Issue number4-5
Publication statusPublished - Jul 1 2000

ASJC Scopus subject areas

  • Developmental Biology
  • Developmental Neuroscience


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