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Defective proteostasis in Alzheimer’s disease
Ageing Research Reviews, 2023The homeostasis of cellular proteins, or proteostasis, is critical for neuronal function and for brain processes, including learning and memory. Increasing evidence indicates that defective proteostasis contributes to the progression of neurodegenerative
Danielle Cozachenco +2 more
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Mitochondria-Associated Proteostasis
Annual Review of Biophysics, 2020Mitochondria are essential organelles in eukaryotes. Most mitochondrial proteins are encoded by the nuclear genome and translated in the cytosol. Nuclear-encoded mitochondrial proteins need to be imported, processed, folded, and assembled into their functional states.
Linhao, Ruan +5 more
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2021
Proteostasis integrates biological pathways controlling biogenesis, trafficking, folding, and degradation of proteins. This book focuses on two protein breakdown/degradation processes (proteolysis), which are part of a normally functioning proteostatic system: the ubiquitin-proteasome system and autophagy.
Niki Chondrogianni +2 more
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Proteostasis integrates biological pathways controlling biogenesis, trafficking, folding, and degradation of proteins. This book focuses on two protein breakdown/degradation processes (proteolysis), which are part of a normally functioning proteostatic system: the ubiquitin-proteasome system and autophagy.
Niki Chondrogianni +2 more
openaire +2 more sources
2021
Abstract Normal cell function and survival depend on carefully regulated synthesis, folding, trafficking, and degradation of proteins. The balance among these processes is referred to as proteostasis. Proteins undergo maturation and folding in the endoplasmic reticulum. This process is error-prone and can be affected by mutations, errors
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Abstract Normal cell function and survival depend on carefully regulated synthesis, folding, trafficking, and degradation of proteins. The balance among these processes is referred to as proteostasis. Proteins undergo maturation and folding in the endoplasmic reticulum. This process is error-prone and can be affected by mutations, errors
openaire +2 more sources
CFTR Folding: From Structure and Proteostasis to Cystic Fibrosis Personalized Medicine
ACS Chemical Biology, 2023Cystic fibrosis (CF) is a lethal genetic disease caused by mutations in the chloride ion channel cystic fibrosis transmembrane conductance regulator (CFTR). Class-II mutants of CFTR lack intermolecular interactions important for CFTR structural stability
E. McDonald, Jens Meiler, Lars Plate
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Nature Medicine, 2015
Accumulation of intracellular damage is an almost universal hallmark of aging. An improved understanding of the systems that contribute to cellular protein quality control has shed light on the reasons for the increased vulnerability of the proteome to stress in aging cells.
Susmita Kaushik, Ana Maria Cuervo
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Accumulation of intracellular damage is an almost universal hallmark of aging. An improved understanding of the systems that contribute to cellular protein quality control has shed light on the reasons for the increased vulnerability of the proteome to stress in aging cells.
Susmita Kaushik, Ana Maria Cuervo
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Proteostasis impairment and ALS
Progress in Biophysics and Molecular Biology, 2022Amyotrophic lateral sclerosis (ALS) is a rapidly progressive and fatal neurodegenerative disease that results from the loss of both upper and lower motor neurons. It is the most common motor neuron disease and currently has no effective treatment. There is mounting evidence to suggest that disturbances in proteostasis play a significant role in ALS ...
Isabella A, Lambert-Smith +2 more
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Cellular Proteostasis in Neurodegeneration
Molecular Neurobiology, 2018The term proteostasis reflects the fine-tuned balance of cellular protein levels, mediated through a vast network of biochemical pathways. This requires the regulated control of protein folding, post-translational modification, and protein degradation.
Alberim Kurtishi +4 more
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Chemical Biology and Drug Design
Neurodegenerative disorders are devastating disorders characterized by gradual loss of neurons and cognition or mobility impairment. The common pathological features of these diseases are associated with the accumulation of misfolded or aggregation of ...
Surbhi Panwar +6 more
semanticscholar +1 more source
Neurodegenerative disorders are devastating disorders characterized by gradual loss of neurons and cognition or mobility impairment. The common pathological features of these diseases are associated with the accumulation of misfolded or aggregation of ...
Surbhi Panwar +6 more
semanticscholar +1 more source