... of the Biochemical Society 2022 extracellular chaperones extracellular proteostasis protein conformation protein misfolding Proteostasis refers to a delicately tuned balance between the processes of protein synthesis, folding, localization, and degradation. Proteins act as sensors, transducers...

...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...

... to populate stable and folded states ( Figure 1 ) but the molecular and phenotypic manifestations of individual mutations can vary, reviewed [ 8 ]. amyotrophic lateral sclerosis molecular chaperones neurodegeneration Parkinson's disease pharmacological chaperones protein misfolding...

... 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...

... and these can be found in Figure 1 . Figure 1 Schematic overview of inflammation-associated triggers which can provoke protein misfolding ER-dependent protein folding in IECs of patients with active IBD is affected by several inflammatory triggers. As mentioned above, different specialized...

..., University of Copenhagen, Universitetsparken 15, 2100 Copenhagen, Denmark 5 1 2009 © The Authors Journal compilation © 2010 Biochemical Society 2010 aging Alzheimer's disease amyloid β-peptide isomerization protein misfolding racemization The word ‘aging’ brings to mind...

... conclusions of the experiment, but also provide a detailed but somewhat different molecular picture of the disruption of the long-range interactions. The loss of protein stability and subsequent protein misfolding and aggregation can have severe consequences (many fatal diseases, such as Alzheimer's...

... and behavioural levels. © 2006 The Biochemical Society 2006 1 To whom correspondence should be addressed (email V.M.O'Connor@soton.ac.uk ). 31 10 2005 genetic disease model organism molecular determinant protein misfolding synapse synaptic function The Biochemical Society...

... 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...