Modulation of heat shock transcription factor 1 as a therapeutic target for small molecule intervention in neurodegenerative disease. [PDF]
PLoS Biology, 2010 Neurodegenerative diseases such as Huntington disease are devastating disorders with no therapeutic approaches to ameliorate the underlying protein misfolding defect inherent to poly-glutamine (polyQ) proteins.
Daniel W Neef +2 more
doaj +1 more source
Pathogenic mutations in the hydrophobic core of the human prion protein can promote structural instability and misfolding [PDF]
, 2010 Transmissible spongiform encephalopathies, or prion diseases, are caused by misfolding and aggregation of the prion protein PrP. These diseases can be hereditary in humans and four of the many disease-associated missense mutants of PrP are in the ...
Daggett, Valerie, van der Kamp, Marc W
core +2 more sources
Neuroprotection targeting protein misfolding on chronic cerebral hypoperfusion in the context of metabolic syndrome [PDF]
, 2018 Metabolic syndrome (MetS) is a cluster of risk factors that lead to microvascular dysfunction and chronic cerebral hypoperfusion (CCH). Long-standing reduction in oxygen and energy supply leads to brain hypoxia and protein misfolding, thereby linking CCH
Capani, Francisco +6 more
core +1 more source
Effect of spermidine on misfolding and interactions of alpha-synuclein. [PDF]
PLoS ONE, 2012 Alpha-synuclein (α-Syn) is a 140 aa presynaptic protein which belongs to a group of natively unfolded proteins that are unstructured in aqueous solutions.
Alexey V Krasnoslobodtsev +5 more
doaj +1 more source
Heat shock factor 1 regulates lifespan as distinct from disease onset in prion disease [PDF]
, 2008 Prion diseases are fatal, transmissible, neurodegenerative diseases caused by the misfolding of the prion protein (PrP). At present, the molecular pathways underlying prion-mediated neurotoxicity are largely unknown.
Aguzzi, Adriano +8 more
core +3 more sources
Impact of translational error‐induced and error‐free misfolding on the rate of protein evolution
Molecular Systems Biology, 2010 What determines the rate of protein evolution is a fundamental question in biology. Recent genomic studies revealed a surprisingly strong anticorrelation between the expression level of a protein and its rate of sequence evolution.
Jian‐Rong Yang +2 more
doaj +1 more source
Matrin3: Disorder and ALS Pathogenesis
Frontiers in Molecular Biosciences, 2022 Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by the degeneration of both upper and lower motor neurons in the brain and spinal cord.
Ahmed Salem +7 more
doaj +1 more source
Systematic interaction network filtering identifies CRMP1 as a novel suppressor of huntingtin misfolding and neurotoxicity [PDF]
, 2015 Assemblies of huntingtin (HTT) fragments with expanded polyglutamine (polyQ) tracts are a pathological hallmark of Huntington's disease (HD). The molecular mechanisms by which these structures are formed and cause neuronal dysfunction and toxicity are ...
Andrade-Navarro, Miguel A. +24 more
core +2 more sources
Role of Protein Misfolding and Proteostasis Deficiency in Protein Misfolding Diseases and Aging
International Journal of Cell Biology, 2013 The misfolding, aggregation, and tissue accumulation of proteins are common events in diverse chronic diseases, known as protein misfolding disorders.
Karina Cuanalo-Contreras +2 more
doaj +1 more source
Divergent effects of PERK and IRE1 signaling on cell viability.
PLoS ONE, 2009 Protein misfolding in the endoplasmic reticulum (ER) activates a set of intracellular signaling pathways, collectively termed the Unfolded Protein Response (UPR). UPR signaling promotes cell survival by reducing misfolded protein levels.
Jonathan H Lin +4 more
doaj +1 more source