منابع مشابه
Protein misfolding and neurodegeneration.
A key molecular pathway implicated in diverse neurodegenerative diseases is the misfolding, aggregation, and accumulation of proteins in the brain. Compelling evidence strongly supports the hypothesis that accumulation of misfolded proteins leads to synaptic dysfunction, neuronal apoptosis, brain damage, and disease. However, the mechanism by which protein misfolding and aggregation trigger neu...
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The crucial event in the development of transmissible spongiform encephalopathies (TSEs) is the conformational change of a host-encoded membrane protein - the cellular PrP(C) - into a disease associated, fibril-forming isoform PrP(Sc). This conformational transition from the alpha-helix-rich cellular form into the mainly beta-sheet containing counterpart initiates an 'autocatalytic' reaction wh...
متن کاملProtein misfolding diseases
Protein misfolding diseases include highly debilitating degenerative disorders like Alzheimer’s and Parkinson’s diseases [1]. The healthcare and financial burden linked to these pathologies has been steadily increasing over the past decade [2]. Actually, there is currently no efficient treatment for misfolding diseases as well as no reliable early diagnostic techniques for them [3]. It is known...
متن کاملProtein Misfolding Diseases.
The majority of protein molecules must fold into defined three-dimensional structures to acquire functional activity. However, protein chains can adopt a multitude of conformational states, and their biologically active conformation is often only marginally stable. Metastable proteins tend to populate misfolded species that are prone to forming toxic aggregates, including soluble oligomers and ...
متن کاملMisfolding Dominates Protein Evolution
The diverse array of protein functions depends upon these molecules’ reliable ability to fold into the native structures determined by their amino-acid sequences. Because mutations that alter a protein’s sequence frequently disrupt its folding, protein evolution explores protein sequence space conservatively, either by point mutations or recombination between related sequences. Attempts to engi...
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ژورنال
عنوان ژورنال: Nature
سال: 2003
ISSN: 0028-0836,1476-4687
DOI: 10.1038/426883a