... system; mtDNA, mitochondrial DNA; OM, outer membrane; OXPHOS, oxidative phosphorylation; SAM, sorting and assembly machinery; TIM, translocase of the inner membrane; TOM, translocase of the outer membrane. mitochondrial dysfunction oxidative stress protein import protein misfolding...

... of the Biochemical Society and distributed under the Creative Commons Attribution License 4.0 (CC BY) . globotriaosylsphingosine lysosomal enzyme proteasome inhibitor protein misfolding transcriptomics Fabry disease (FD, OMIM 301500) is one of more than 40 lysosomal storage diseases (LSD) [ 1...

...). heat shock molecular chaperones protein misfolding protein quality control serine proteases β-barrel outer membrane proteins Bacterial cells often face a wide array of environmental stresses, including UV irradiation, heat shock, low pH, oxidative stress, and osmotic shock, which may...

...@chemistry.iitd.ac.in ) or Bishwajit Kundu ( bkundu@bioschool.iitd.ac.in ) 6 8 2016 25 10 2016 31 10 2016 1 11 2016 © 2017 The Author(s); published by Portland Press Limited on behalf of the Biochemical Society 2017 amyloid bispidine cytotoxicity oligomer prion protein misfolding...

... potential in AD treatment. Alzheimer's disease amyloid-β model organisms molecular chaperones protein misfolding Correspondence: Jenny Presto ( jenny.presto@ki.se ); Jan Johansson ( janne.johansson@ki.se ) 1 4 2016 9 8 2016 11 8 2016 11 8 2016 © 2016 The Author(s...

... there is activation of the UPR (unfolded protein response) as a result of misfolded protein retention. As a positive control for protein misfolding, we also expressed the mutant G221R neuroligin3, which is known to be completely retained within the ER. Our data show that overexpression of either R451C or G221R mutant...

... (Miltenyi Biotec), anti-mRFP (Chromotek) and anti-HA (Roche) antibodies. cell biology endoplasmic reticulum endoplasmic-reticulum-associated degradation (ERAD) glycobiology glycoprotein glycosylation protein degradation protein misfolding The endoplasmic reticulum (ER) is the major...

... formation of toxic Aβ-species as well as the self-templating activity of Aβ fibrils, properties that could have therapeutic utility. Alzheimer’s disease amyloid Aβ42 (amyloid-β 42) Aβ43 (amyloid-β 43) foldamer protein misfolding Protein misfolding can be fatal [ 1 , 2 ]. Proteins misfold...

... by protein misfolding if the proteasome is down-regulated as recently reported in humans with Type 2 diabetes. It remains unknown, however, whether the glucose sensor enzyme glucokinase is involved in this process. A direct interaction between glucokinase and ubiquitin could be shown in vivo by FRET...

... protein (PrP) protein aggregation protein misfolding Genetic prion diseases have a pattern of autosomal dominant inheritance and are linked to point mutations or insertions in the PRNP gene encoding the cellular prion protein (PrP C ) on chromosome 20 [ 1 ]. More than 20 point mutations have...

... Biochemical Society 2012 BRICHOS domain HET-S prion hydrophobin melanin synthesis proinsulin C-peptide protein misfolding self-chaperoning spider silk For every polypeptide chain, a specific conformation or ensemble of conformations exists that represents its native fold. Yet, many...

... ganglioside membrane bilayer protein misfolding surface plasmon field-enhanced fluorescence spectroscopy Aβ peptides require treatment to ensure disaggregation and removal of preaggregated species [ 11 ], Solvents including HFIP, DMSO and TFE (trifluroethanol) have been shown to significantly...

... amyloidotic polyneuropathy and senile systemic amyloidosis. Although it has been widely accepted that protein misfolding of the monomeric form of TTR is a rate-limiting step for amyloid formation, no effective therapy targeting this misfolding step is available. In the present study, we focused on CyDs...

... protein misfolding Prion diseases are invariably fatal neurodegenerative disorders of humans and animals that arise because of misfolding of the PrP C [cellular isoform of PrP (prion protein)], a GPI (glycosylphosphatidylinositol)-anchored glycoprotein whose function is not clear [ 1 ]. Unique...

...-oxoglutarate dehydrogenase post-translational modification protein misfolding protein–protein interaction substrate channelling The 2-oxo acid dehydrogenase multienzyme complexes play pivotal roles in metabolism. They catalyse the oxidative decarboxylation of 2-oxo acids, generating NADH...

... Society 2007 cellular chaperone conformational disease cystic fibrosis cystic fibrosis transmembrane conductance regulator (CFTR) endoplasmic-reticulum-associated degradation (ERAD) protein folding protein misfolding pharmacological chaperone Biological organisms provide a unique...

...-specific chaperone (ASSC) 1-deoxygalactonojirimycin (DGJ) endoplasmic reticulum-associated degradation (ERAD) Fabry disease α-galactosidase A protein misfolding 1 To whom correspondence should be addressed (email jian-qiang.fan@mssm.edu ). 5 4 2007 24 5 2007 8 6 2007...