Although a number of features distinguish the disease isoform of the prion protein (PrPSc) from its normal cellular counterpart (PrPC) in the transmissible spongiform encephalopathies (TSEs), the neuropathogenesis of these diseases remains an enigma. The amyloid fibrils formed by fragments of human PrP have, however, been shown to be directly neurotoxic in vitro. We show here that sulphated polysaccharides (heparin, keratan and chondroitin) inhibit the neurotoxicity of these amyloid fibrils and this appears to be mediated via inhibition of the polymerization of the PrP peptide into fibrils. This provides a rationale for the therapeutic effects of sulphated polysaccharides and suggests a rapid in vitro functional screen for TSE therapeutics.
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October 1998
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Research Article|
October 15 1998
Sulphated glycosaminoglycans prevent the neurotoxicity of a human prion protein fragment
Mar PÉREZ;
Mar PÉREZ
1Centro de Biología Molecular, Universidad Autónoma de Madrid, 28049-Madrid, Spain
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Francisco WANDOSELL;
Francisco WANDOSELL
1Centro de Biología Molecular, Universidad Autónoma de Madrid, 28049-Madrid, Spain
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Camilo COLAÇO;
Camilo COLAÇO
1
1Centro de Biología Molecular, Universidad Autónoma de Madrid, 28049-Madrid, Spain
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Jesús AVILA
Jesús AVILA
2
1Centro de Biología Molecular, Universidad Autónoma de Madrid, 28049-Madrid, Spain
2To whom correspondence should be addressed (e-mail javila@cbm.uam.es).
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Publisher: Portland Press Ltd
Received:
March 23 1998
Revision Received:
July 03 1998
Accepted:
August 11 1998
Online ISSN: 1470-8728
Print ISSN: 0264-6021
The Biochemical Society, London © 1998
1998
Biochem J (1998) 335 (2): 369–374.
Article history
Received:
March 23 1998
Revision Received:
July 03 1998
Accepted:
August 11 1998
Citation
Mar PÉREZ, Francisco WANDOSELL, Camilo COLAÇO, Jesús AVILA; Sulphated glycosaminoglycans prevent the neurotoxicity of a human prion protein fragment. Biochem J 15 October 1998; 335 (2): 369–374. doi: https://doi.org/10.1042/bj3350369
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