Neurodegenerative disease Amyloid pores from pathogenic mutations

Alzheimer's and Parkinson's diseases are associated with the formation in the brain of amyloid fibrils from β-amyloid and α-synuclein proteins, respectively. It is likely that oligomeric fibrillization intermediates (protofibrils), rather than the fibrils themselves, are pathogenic, but th...

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Veröffentlicht in:Nature (London) 2002-07, Vol.418 (6895), p.291-291
Hauptverfasser: Lansbury, Peter T, Lashuel, Hilal A, Hartley, Dean, Petre, Benjamin M, Walz, Thomas
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Sprache:eng
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Zusammenfassung:Alzheimer's and Parkinson's diseases are associated with the formation in the brain of amyloid fibrils from β-amyloid and α-synuclein proteins, respectively. It is likely that oligomeric fibrillization intermediates (protofibrils), rather than the fibrils themselves, are pathogenic, but the mechanism by which they cause neuronal death remains a mystery. We show here that mutant amyloid proteins associated with familial Alzheimer's and Parkinson's diseases form morphologically indistinguishable annular protofibrils that resemble a class of pore-forming bacterial toxins, suggesting that inappropriate membrane permeabilization might be the cause of cell dysfunction and even cell death in amyloid diseases.
ISSN:0028-0836
1476-4687
DOI:10.1038/418291a