..., and inflammation to restore homeostasis. Lastly, we highlight outstanding questions to guide future research in this field. extracellular chaperones extracellular proteostasis protein conformation protein misfolding Proteostasis refers to a delicately tuned balance between the processes of protein...

...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...

... the evidence for ubiquitination in this critical pathway, the precise complement of ubiquitin ligases and deubiquitinases that modulate this process remains under investigation. Whilst chaperones act as the first line of defence against protein misfolding, the ubiquitination machinery has a pivotal role...

... are not specific but involve high-affinity binding to hydrophobic residues including SOD1 β-strand 7. amyotrophic lateral sclerosis molecular chaperones neurodegeneration Parkinson's disease pharmacological chaperones protein misfolding The central dogma of molecular biology dictates...

... Alzheimer's disease amyloid insulin/IGF-1-like signalling (IIS) protein misfolding tau AD and all of the age-related neurodegenerative disorders are fundamentally protein-folding disorders. In AD, excessive levels of a peptide, Aβ (amyloid β-peptide), and a protein, the MAPT (microtubule...

... compilation © 2011 Biochemical Society 2011 endoplasmic reticulum stress (ER stress) inflammatory bowel disease (IBD) intestinal epithelial cell protein misfolding unfolded protein response (UPR) xenobiotic Chaperones and heat-shock proteins mediate protein folding inside the ER. For example...

..., such as soluble Aβ oligomers and protofibrils, which accumulate in the synapses, producing a dramatic impairment of synaptic plasticity [ 17 , 18 ]. The aggregation of Aβ is not the only neuropathological event of AD caused by protein misfolding; another example is the polymerization of the microtubule-associated...

... 2007 Molecular Dynamics protein lysozyme protein misfolding protein stability single mutation wavelet analysis The loss of protein stability and subsequent protein misfolding and aggregation can have severe consequences (many fatal diseases, such as Alzheimer's disease, are associated...

... The Biochemical Society 2006 1 To whom correspondence should be addressed (email V.M.O'[email protected] ). genetic disease model organism molecular determinant protein misfolding synapse synaptic function The Biochemical Society focused meeting ‘Molecular Determinants of Synaptic...

... disease protein misfolding scrapie CJD, Creutzfeldt-Jakob disease TSE, transmissible spongiform encephalopathy PMCA, protein misfolding cyclic amplification Amyloidogenic Proteins Involved in Neurodegeneration and Therapeutic Implications 32 33 34 35 36 37 38 39 Gabizon, R., Meiner, Z...