...Sashary Ramos; Jennifer C. Lee Neurodegenerative diseases, such as Alzheimer's and Parkinson's, share a common pathological feature of amyloid structure accumulation. However, the structure-function relationship between these well-ordered, β-sheet-rich, filamentous protein deposits and disease...

... (CPR-AFM) methodology and its application in 3D reconstruction of individual helical amyloid filaments in the context of the challenges presented by the structural analysis of highly polymorphous and heterogeneous amyloid protein structures. How individual particle-level structural analysis can...

...Pernilla Wittung-Stafshede Toxic aggregation of proteins and peptides into amyloid fibers is the basis of several human diseases. In each disease, a particular peptide noncovalently assembles into long thin structures with an overall cross-β fold. Amyloids are not only related to disease...

...Marcus Fändrich; Matthias Schmidt Systemic amyloidosis is defined as a protein misfolding disease in which the amyloid is not necessarily deposited within the same organ that produces the fibril precursor protein. There are different types of systemic amyloidosis, depending on the protein...

.... Redox signalling has been shown to play roles in almost every step of protein aggregation and amyloid formation, from aggregation initiation to the rapid oligomerization of large amyloids, which tend to be less toxic than oligomeric prefibrillar aggregates. We explore the hypothesis that age-associated...

...) . amyloid mitochondria mitophagy neurodegeneration tau proteins α-synuclein The energetic requirements of neuronal excitability, synaptic activity and plasticity are extensive and are almost exclusively fulfilled by mitochondrial oxidative phosphorylation (OXPHOS) [ 1 ]. Mitochondria are often...

... elusive, as they seem to adopt different functions dependent on disease progression and the extent of accompanying parenchymal inflammation. Astrocytes may contribute to the clearance of amyloid β-peptide (Aβ) and restrict the spread of inflammation in the brain. Conversely, they may contribute...

...Plamena R. Angelova; Andrey Y. Abramov Alzheimer's disease (AD) is a neurodegenerative disease characterized by the aggregation of amyloid β-peptide (Aβ) into β-sheet-rich fibrils. Although plaques containing Aβ fibrils have been viewed as the conventional hallmark of AD, recent research implicates...

... Biochemical Society 2013 amyloid Gaucher disease glucocerebrosidase lysosome Parkinson’s disease α-synuclein This work was supported by the Intramural Research Program of the National Heart, Lung, and Blood Institute of the National Institutes of Health. References 1 Butters T.D...

...., 26–27 March 2012. Organized and Edited by Jennifer Potts (York, U.K.) and Mike Williamson (Sheffield, U.K.). BOG octyl β- D -glucopyranoside HSQC heteronuclear single-quantum coherence NAC non-amyloid-β component SEC size-exclusion chromatography We thank Dr Linda...

... be addressed to either of these authors (email [email protected] or [email protected] ). 10 7 2012 © The Authors Journal compilation © 2012 Biochemical Society 2012 amyloid chaperone evolution protein aggregation During evolution, cells have been equipped...

... and fibrils of the Aβ (amyloid β-peptide) and tau protein accumulate in specific brain regions. This is associated with the progressive destruction of synaptic circuits controlling memory and higher mental function. The primary signalling mechanisms that (i) become defective in AD to alter the normal...

...Li-Jun Chen; Elizabeth B. Sawyer; Sarah Perrett Ure2, a regulator of nitrogen metabolism, is the protein determinant of the [ URE3 ] prion state in Saccharomyces cerevisiae . Upon conversion into the prion form, Ure2 undergoes a heritable conformational change to an amyloid-like aggregated state...

...Jean-Pierre Brion; Kunie Ando; Céline Heraud; Karelle Leroy NFTs (neurofibrillary tangles) in Alzheimer's disease and in tauopathies are hallmark neuropathological lesions whose relationship with neuronal dysfunction, neuronal death and with other lesions [such as Aβ (amyloid β-peptide) pathology...

... and amyloid in this disease. Oligomeric and aggregated forms of the Aβ (amyloid β-peptide) and hyperphosphorylated and filamentous forms of the microtubule-associated protein tau are the precursors of neuritic plaques and neurofibrillary tangles, the pathological hallmarks of AD (Alzheimer's disease...

... are able to take on conformations other than their native fold to form long fibres known as amyloid. In vivo , these are associated with misfolding diseases, such as Alzheimer's disease, Type 2 diabetes and the amyloidoses. In vitro , peptide assembly leads to amyloid-like fibres that have high stability...

...David Allsop; Jennifer Mayes; Susan Moore; Atef Masad; Brian J. Tabner Using a method based on ESR spectroscopy and spin-trapping, we have shown that Aβ (amyloid β-peptide) (implicated in Alzheimer's disease), α-synuclein (implicated in Parkinson's disease), ABri (British dementia peptide...

... of amyloid proteins involved in neurodegenerative diseases. It is anticipated that the use of SSNMR in drug discovery will become more widespread in the wake of advances in hardware and methodological developments. All amyloid fibrils contain repeat arrays of β-strands arranged with interstrand hydrogen...

...C. Dingwall The amyloidogenic processing pathway of the APP (amyloid precursor protein) generates Aβ (amyloid β-peptide), the major constituent in Alzheimer's disease senile plaques. This processing is catalysed by two unusual membrane-localized aspartic proteinases, β-secretase [BACE1 (β-site APP...

... cells transfected with α-synuclein (A53T), a familial PD-associated mutation. 1 To whom correspondence should be addressed (email [email protected] ). 10 8 2005 © 2005 The Biochemical Society 2005 amyloid fibril Parkinson's disease β-sheet-breaker peptide α-synuclein...

... are present, but in the absence of mature amyloid fibrils. The generation of ROS during early-stage protein aggregation may be a common, fundamental molecular mechanism underlying the pathogenesis of oxidative damage, neurodegeneration and cell death in several different neurodegenerative diseases. Drugs...

...M.N. Pangalos; S.J. Jacobsen; P.H. Reinhart AD (Alzheimer's disease) is characterized neuropathologically by the presence of amyloid plaques, neurofibrillary tangles and profound grey matter loss. The ‘amyloid’ hypothesis postulates that the toxic Aβ (amyloid β) peptide, enzymatically derived from...

... the neurotoxic Alzheimer's amyloid peptide. The only known homologue of ACE, termed ACE2, is critical to cardiovascular function, but its physiological substrates and precise metabolic roles remain to be elucidated. Other members of these growing metallopeptidase families await further characterization...

...O. M. A. EI-Agnaf; G. B. Irvine Fibrillar deposits of α-synuclein occur in several neurodegenerative diseases. Two mutant forms of α-synuclein have been associated with early-onset Parkinson's disease, and a fragment has been identified as the non-amyloid-β peptide component of Alzheimer's disease...

...W. E. Müller; C. Kirsch; G. P. Eckert β-Amyloid (Aβ) protein is the major constituent of senile plaques and cerebrovascular deposits characteristic of Alzheimer's disease (AD). The causal relationship between Aβ and AD-specific lesions like neurodegeneration and atrophy is still not known...