Protein Misfolding and Human Disease by Christopher Dobson

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Lipid Oxidation at the Crossroads: Oxidative Stress and Neurodegeneration Explored in <i>Caenorhabditis elegans</i> by Julia Tortajada-Pérez, Andrea del Valle Carranza, Cristina Trujillo-del Río, Mar Collado-Pérez, José María Millán, Gema García-García, Rafael Pascual Vázquez-Manrique

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Chronic Gastroesophageal Reflux Dysregulates Proteostasis in Esophageal Epithelial CellsSummary by Kodisundaram Paulrasu, Ravindran Caspa Gokulan, Wael El-Rifai, Zhibin Chen, Jianwen Que, Timothy C. Wang, Olivier G. Boutaud, Karoline Briegel, Sergey I. Dikalov, Monica T. Garcia-Buitrago, Alexander I. Zaika

“…Here, we investigated the interrelationship between isoLG adduction and aggregation of cellular proteins. Methods: Protein misfolding and aggregation were analyzed using multiple protein misfolding and aggregation assays. …”
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Interaction between 𝛼-Synuclein and Other Proteins in Neurodegenerative Disorders by Kurt A. Jellinger

“…Despite clinical, genetic, and experimental differences, evidence increasingly indicates considerable overlap between synucleinopathies and tauopathies or other protein-misfolding diseases. Inclusions, characteristics of these disorders, also occurring in other neurodegenerative diseases, suggest interactions of pathological proteins engaging common downstream pathways. …”
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Mechanisms of Neuroprotection by Protein Disulphide Isomerase in Amyotrophic Lateral Sclerosis by Adam K. Walker, Julie D. Atkin

“…Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease characterised by the progressive loss of motor neurons, leading to paralysis and death within several years of onset. Although protein misfolding is a key feature of ALS, the upstream triggers of disease remain elusive. …”
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Altered Gene Expression, Mitochondrial Damage and Oxidative Stress: Converging Routes in Motor Neuron Degeneration by Luisa Rossi, Cristiana Valle, Maria Teresa Carrì

“…At the cellular level, MNDs share features such as protein misfolding and aggregation, mitochondrial damage and energy deficit, and excitotoxicity and calcium mishandling. …”
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Hsp104 suppresses polyglutamine-induced degeneration post onset in a drosophila MJD/SCA3 model. by Mimi Cushman-Nick, Nancy M Bonini, James Shorter

“…There are no effective therapeutics that antagonize or reverse the protein-misfolding events underpinning polyglutamine (PolyQ) disorders, including Spinocerebellar Ataxia Type-3 (SCA3). …”
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Inotersen to Treat Polyneuropathy Associated with Hereditary Transthyretin (hATTR) Amyloidosis by Christopher Robinson, Cynthia Pham, Alec M. Zamarripa, Chase S. Dugay, Christopher A. Lee, Amnon A. Berger, Avi Landman, Elyse M. Cornett, Hisham Kassem, Alan D. Kaye, Ivan Urits, Omar Viswanath, Latha Ganti

“…# Background Amyloidosis is a group of diseases with the common pathophysiology of protein misfolding and aberrant deposition in tissue. There are both acquired and hereditary forms of this disease, and this review focuses on the latter hereditary transthyretin-mediated (hATTR). hATTR affects about 50,000 individuals globally and mostly appears as one of three syndromes - cardiac, polyneuropathy, and oculoleptomeningeal. …”
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Dysregulation of the Autophagy-Endolysosomal System in Amyotrophic Lateral Sclerosis and Related Motor Neuron Diseases by Asako Otomo, Lei Pan, Shinji Hadano

“…More than 16 causative genes for ALS/MNDs have been identified and their underlying pathogenesis, including oxidative stress, endoplasmic reticulum stress, excitotoxicity, mitochondrial dysfunction, neural inflammation, protein misfolding and accumulation, dysfunctional intracellular trafficking, abnormal RNA processing, and noncell-autonomous damage, has begun to emerge. …”
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Enhanced detection of chronic wasting disease in muscle tissue harvested from infected white-tailed deer employing combined prion amplification assays. by Caitlyn N Kraft, David W Bissinger, Erin E McNulty, Nathaniel D Denkers, Candace K Mathiason

“…Here we combined two prion amplification assays, the product of protein misfolding cyclic amplification (PMCA) applied directly into real-time quaking induced conversion (RT-QuIC) [denoted now as PQ] to demonstrate the presence of prion seeding activity (i.e. prions) in ~55% of hamstring muscles harvested from CWD-positive white-tailed deer. …”
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Hyperthermia and targeting heat shock proteins: innovative approaches for neurodegenerative disorders and Long COVID by David M. Smadja, David M. Smadja, M. Marc Abreu, M. Marc Abreu

“…Neurodegenerative diseases (NDs) and Long COVID represent critical and growing global health challenges, characterized by complex pathophysiological mechanisms including neuronal deterioration, protein misfolding, and persistent neuroinflammation. The emergence of innovative therapeutic approaches, such as whole-body hyperthermia (WBH), offers promising potential to modulate underlying pathophysiological mechanisms in NDs and related conditions like Long COVID. …”
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Proteostasis and Mitochondrial Role on Psychiatric and Neurodegenerative Disorders: Current Perspectives by Pablo Olivero, Carlo Lozano, Ramón Sotomayor-Zárate, Nicolás Meza-Concha, Marcelo Arancibia, Claudio Córdova, Wilfredo González-Arriagada, Ricardo Ramírez-Barrantes, Ivanny Marchant

“…Proteostasis involves processes that are fundamental for neural viability. Thus, protein misfolding and the formation of toxic aggregates at neural level, secondary to dysregulation of the conservative mechanisms of proteostasis, are associated with several neuropsychiatric conditions. …”
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Copper homeostasis and neurodegenerative diseases by Yuanyuan Wang, Daidi Li, Kaifei Xu, Guoqing Wang, Feng Zhang

“…By synthesizing the current findings on the functions of copper in oxidative stress, neuroinflammation, mitochondrial dysfunction, and protein misfolding, we aim to elucidate the mechanisms by which copper contributes to a wide range of hereditary and neuronal disorders, such as Wilson’s disease, Menkes’ disease, Alzheimer’s disease, Parkinson’s disease, amyotrophic lateral sclerosis, Huntington’s disease, and multiple sclerosis. …”
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Anthracyclines disaggregate and restore mutant p63 function: a potential therapeutic approach for AEC syndrome by Fabiana Boncimino, Ludovica D’Auria, Kristina Todorova, Sabina Y. van der Zanden, Jacques Neefjes, Anna Mandinova, Caterina Missero, Stefano Sol

“…Our previous studies demonstrated that mutations associated with AEC syndrome lead to p63 protein misfolding and aggregation, exerting a dominant-negative effect. …”
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Autoinflammatory diseases. Part 1: concept, classification, immunobiology, diagnosis by O.V. Shvaratska, M.V. Kalichevska, O.V. Klymenko, O.M. Taran, O.V. Klimova, Y.V. Vilenskyi, T.M. Plekhanova, T.K. Mavropulo

“…Based on the underlying molecular pathogenesis, the following classification categories of SAIDs have been suggested recently: inflammasomopathies and other enhanced IL-1 signaling syndromes, interferonopathies, relopathies, protein misfolding diseases/endoplasmic reticulum stress syndromes, other cytokine-signaling disorders and complementopathies. …”
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N88S seipin-related seipinopathy is a lipidopathy associated with loss of iron homeostasis by Mariana O. Ribeiro, Mafalda Oliveira, Verónica Nogueira, Vítor Costa, Vitor Teixeira

“…Conclusions The study highlights that the effects of N88S seipin mutation extend beyond protein misfolding, with significant disruptions in lipid metabolism and iron homeostasis. …”
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Proteostasis Decline and Redox Imbalance in Age-Related Diseases: The Therapeutic Potential of NRF2 by Brigitta Buttari, Antonella Tramutola, Ana I. Rojo, Niki Chondrogianni, Sarmistha Saha, Alessandra Berry, Letizia Giona, Joana P. Miranda, Elisabetta Profumo, Sergio Davinelli, Andreas Daiber, Antonio Cuadrado, Fabio Di Domenico

“…By inducing NRF2, it is possible to mitigate the damaging effects of oxidative stress, metabolic dysfunction, and inflammation, thus reducing protein misfolding. The review highlights NRF2’s therapeutic implications for neurodegenerative diseases and cardiovascular conditions, emphasizing its role in improving proteostasis and redox homeostasis Additionally, it summarizes current research into NRF2 as a therapeutic target, offering hope for innovative treatments to counteract the effects of aging and associated diseases.…”
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