PubMed 12124613

Referenced in Channelpedia wiki pages of: none

Automatically associated channels: Kv2.1

Title: Neurodegenerative disease: amyloid pores from pathogenic mutations.

Authors: Hilal A Lashuel, Dean Hartley, Benjamin M Petre, Thomas Walz, Peter T Lansbury

Journal, date & volume: Nature, 2002 Jul 18 , 418, 291

PubMed link:

Alzheimer's and Parkinson's diseases are associated with the formation in the brain of amyloid fibrils from beta-amyloid and alpha-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.