Molecular mechanisms of proteinopathies across neurodegenerative disease: a review
Neurological Research and Practice, 2019 Background Although there is a range of different symptoms across neurodegenerative diseases, they have been noted to have common pathogenic features.
Alexander P. Marsh
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Protein folding disorders: Toward a basic biological paradigm [PDF]
, 2010 Mechanistic 'physics' models of protein folding fail to account for the observed spectrum of protein folding and aggregation disorders, suggesting that a more appropriately biological paradigm will be needed for understanding the etiology ...
Rodrick Wallace
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Nanomedicine and protein misfolding diseases [PDF]
Nanomedicine: Nanotechnology, Biology and Medicine, 2005 Misfolding and self assembly of proteins in nano-aggregates of different sizes and morphologies (nano-ensembles, primarily nanofilaments and nano-rings) is a complex phenomenon that can be facilitated, impeded, or prevented, by interactions with various intracellular metabolites, intracellular nanomachines controlling protein folding and interactions ...
Alexey V, Kransnoslobodtsev +5 more
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Impaired Cleavage of Preproinsulin Signal Peptide Linked to Autosomal-Dominant Diabetes [PDF]
, 2012 Recently, missense mutations upstream of preproinsulin’s signal peptide (SP) cleavage site were reported to cause mutant INS gene-induced diabetes of youth (MIDY).
Arvan, Peter +8 more
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Methionine 129 variant of human prion protein oligomerizes more rapidly than the valine 129 variant - Implications for disease susceptibility to Creutzfeldt-Jakob disease [PDF]
, 2004 The human PrP gene (PRNP) has two common alleles that encode either methionine or valine at codon 129. This polymorphism modulates disease susceptibility and phenotype of human transmissible spongiform encyphalopathies, but the molecular mechanism by ...
Abdessamad Tahiri-Alaoui +56 more
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Nature Communications, 2017 Huntington’s disease (HD) is caused by misfolding of mutant Htt protein. The authors find that in HD models, the decreased expression of heat shock transcription factor 1 that usually protects against protein misfolding, is in part caused by elevated ...
Rocio Gomez-Pastor +12 more
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Estrogens promote misfolded proinsulin degradation to protect insulin production and delay diabetes [PDF]
, 2018 Summary: Conjugated estrogens (CE) delay the onset of type 2 diabetes (T2D) in postmenopausal women, but the mechanism is unclear. In T2D, the endoplasmic reticulum (ER) fails to promote proinsulin folding and, in failing to do so, promotes ER stress and
Allard, Camille +12 more
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Theoretical model of prion propagation: A misfolded protein induces misfolding [PDF]
Proceedings of the National Academy of Sciences, 2005 There is a hypothesis that dangerous diseases such as bovine spongiform encephalopathy, Creutzfeldt-Jakob, Alzheimer's, fatal familial insomnia, and several others are induced by propagation of wrong or misfolded conformations of some vital proteins. If for some reason the misfolded conformations were acquired by many such protein molecules it might ...
Edyta, Małolepsza +3 more
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Extragenic Suppression analysis of TS mutations using Sec61p [PDF]
, 2007 During synthesis, secretory and membrane proteins are cotranslationally translocated into the lumen of the endoplasmic reticulum through an aqueous gated channel.
Sterling Smith
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Interaction of tau with the RNA-Binding Protein TIA1 Regulates tau Pathophysiology and Toxicity
Cell Reports, 2016 Summary: Dendritic mislocalization of microtubule associated protein tau is a hallmark of tauopathies, but the role of dendritic tau is unknown. We now report that tau interacts with the RNA-binding protein (RBP) TIA1 in brain tissue, and we present the ...
Tara Vanderweyde +15 more
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