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A protease-resistant 61-residue prion peptide causes neurodegeneration in transgenic mice.
Supattapone, S; Bouzamondo, E; Ball, H L; Wille, H; Nguyen, H O; Cohen, F E; DeArmond, S J; Prusiner, S B; Scott, M.
Afiliação
  • Supattapone S; Institute for Neurodegenerative Diseases, University of California at San Francisco, San Francisco, California 94143, USA.
Mol Cell Biol ; 21(7): 2608-16, 2001 Apr.
Article em En | MEDLINE | ID: mdl-11259607
ABSTRACT
An abridged prion protein (PrP) molecule of 106 amino acids, designated PrP106, is capable of forming infectious miniprions in transgenic mice (S. Supattapone, P. Bosque, T. Muramoto, H. Wille, C. Aagaard, D. Peretz, H.-O. B. Nguyen, C. Heinrich, M. Torchia, J. Safar, F. E. Cohen, S. J. DeArmond, S. B. Prusiner, and M. Scott, Cell 96869-878, 1999). We removed additional sequences from PrP106 and identified a 61-residue peptide, designated PrP61, that spontaneously adopted a protease-resistant conformation in neuroblastoma cells. Synthetic PrP61 bearing a carboxy-terminal lipid moiety polymerized into protease-resistant, beta-sheet-enriched amyloid fibrils at a physiological salt concentration. Transgenic mice expressing low levels of PrP61 died spontaneously with ataxia. Neuropathological examination revealed accumulation of protease-resistant PrP61 within neuronal dendrites and cell bodies, apparently causing apoptosis. PrP61 may be a useful model for deciphering the mechanism by which PrP molecules acquire protease resistance and become neurotoxic.
Assuntos

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Príons / Camundongos Transgênicos / Doenças Neurodegenerativas Tipo de estudo: Etiology_studies Limite: Animals Idioma: En Ano de publicação: 2001 Tipo de documento: Article

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Príons / Camundongos Transgênicos / Doenças Neurodegenerativas Tipo de estudo: Etiology_studies Limite: Animals Idioma: En Ano de publicação: 2001 Tipo de documento: Article