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Proteostasis

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https://www.readbyqxmd.com/read/28549133/drug-repurposing-to-target-proteostasis-and-prevent-neurodegeneration-accelerating-translational-efforts
#1
Gabriela Mercado, Claudio Hetz
No abstract text is available yet for this article.
June 1, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28545550/genetic-variation-in-neurodegenerative-diseases-and-its-accessibility-in-the-model-organism-caenorhabditis-elegans
#2
REVIEW
Yiru Anning Wang, Jan Edward Kammenga, Simon Crawford Harvey
BACKGROUND: Neurodegenerative diseases (NGDs) such as Alzheimer's and Parkinson's are debilitating and largely untreatable conditions strongly linked to age. The clinical, neuropathological, and genetic components of NGDs indicate that neurodegeneration is a complex trait determined by multiple genes and by the environment. MAIN BODY: The symptoms of NGDs differ among individuals due to their genetic background, and this variation affects the onset and progression of NGD and NGD-like states...
May 25, 2017: Human Genomics
https://www.readbyqxmd.com/read/28544880/systems-wide-studies-uncover-commander-a-multiprotein-complex-essential-to-human-development
#3
REVIEW
Anna L Mallam, Edward M Marcotte
Recent mass spectrometry maps of the human interactome independently support the existence of a large multiprotein complex, dubbed "Commander." Broadly conserved across animals and ubiquitously expressed in nearly every human cell type examined thus far, Commander likely plays a fundamental cellular function, akin to other ubiquitous machines involved in expression, proteostasis, and trafficking. Experiments on individual subunits support roles in endosomal protein sorting, including the trafficking of Notch proteins, copper transporters, and lipoprotein receptors...
May 24, 2017: Cell Systems
https://www.readbyqxmd.com/read/28539871/protein-quality-control-and-the-amyotrophic-lateral-sclerosis-frontotemporal-dementia-continuum
#4
Hamideh Shahheydari, Audrey Ragagnin, Adam K Walker, Reka P Toth, Marta Vidal, Cyril J Jagaraj, Emma R Perri, Anna Konopka, Jessica M Sultana, Julie D Atkin
Protein homeostasis, or proteostasis, has an important regulatory role in cellular function. Protein quality control mechanisms, including protein folding and protein degradation processes, have a crucial function in post-mitotic neurons. Cellular protein quality control relies on multiple strategies, including molecular chaperones, autophagy, the ubiquitin proteasome system, endoplasmic reticulum (ER)-associated degradation (ERAD) and the formation of stress granules (SGs), to regulate proteostasis. Neurodegenerative diseases are characterized by the presence of misfolded protein aggregates, implying that protein quality control mechanisms are dysfunctional in these conditions...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28535259/altered-organisation-of-the-intermediate-filament-cytoskeleton-and-relocalisation-of-proteostasis-modulators-in-cells-lacking-the-ataxia-protein-sacsin
#5
Emma J Duncan, Roxanne Larivière, Teisha Y Bradshaw, Fabiana Longo, Nicolas Sgarioto, Matthew J Hayes, Lisa E L Romano, Suran Nethisinghe, Paola Giunti, Michaela B Bruntraeger, Heather D Durham, Bernard Brais, Francesca Maltecca, Benoit J Gentil, J Paul Chapple
Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (ARSACS) is caused by mutations in the gene SACS, encoding the 520 kDa protein sacsin. Although sacsin's physiological role is largely unknown, its sequence domains suggest a molecular chaperone or protein quality control function. Consequences of its loss include neurofilament network abnormalities, specifically accumulation and bundling of perikaryal and dendritic neurofilaments.To investigate if loss of sacsin affects intermediate filaments more generally, the distribution of vimentin was analysed in ARSACS patient fibroblasts and in cells where sacsin expression was reduced...
May 23, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28528489/the-ribosome-bound-quality-control-complex-from-aberrant-peptide-clearance-to-proteostasis-maintenance
#6
REVIEW
Quentin Defenouillère, Micheline Fromont-Racine
Proteostasis in eukaryotes is maintained by compartment-specific quality control pathways, which enable the refolding or the degradation of defective polypeptides to prevent the toxicity that may arise from their aggregation. Among these processes, translational protein quality control is performed by the Ribosome-bound Quality Control complex (RQC), which recognizes nascent peptides translated from aberrant mRNAs, polyubiquitylates these aberrant peptides, extracts them from the stalled 60S subunit and finally escorts them to the proteasome for degradation...
May 20, 2017: Current Genetics
https://www.readbyqxmd.com/read/28512398/protein-homeostasis-in-amyotrophic-lateral-sclerosis-therapeutic-opportunities
#7
REVIEW
Christopher P Webster, Emma F Smith, Pamela J Shaw, Kurt J De Vos
Protein homeostasis (proteostasis), the correct balance between production and degradation of proteins, is essential for the health and survival of cells. Proteostasis requires an intricate network of protein quality control pathways (the proteostasis network) that work to prevent protein aggregation and maintain proteome health throughout the lifespan of the cell. Collapse of proteostasis has been implicated in the etiology of a number of neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS), the most common adult onset motor neuron disorder...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28510050/a-review-of-multi-domain-and-flexible-molecular-chaperones-studies-by-small-angle-x-ray-scattering
#8
REVIEW
Júlio C Borges, Thiago V Seraphim, Paulo R Dores-Silva, Leandro R S Barbosa
Intrinsic flexibility is closely related to protein function, and a plethora of important regulatory proteins have been found to be flexible, multi-domain or even intrinsically disordered. On the one hand, understanding such systems depends on how these proteins behave in solution. On the other, small-angle X-ray scattering (SAXS) is a technique that fulfills the requirements to study protein structure and dynamics relatively quickly with few experimental limitations. Molecular chaperones from Hsp70 and Hsp90 families are multi-domain proteins containing flexible and/or disordered regions that play central roles in cellular proteostasis...
June 2016: Biophysical Reviews
https://www.readbyqxmd.com/read/28503130/uncoupling-the-trade-off-between-somatic-proteostasis-and-reproduction-in-caenorhabditis-elegans-models-of-polyglutamine-diseases
#9
Netta Shemesh, Nadav Shai, Lana Meshnik, Rotem Katalan, Anat Ben-Zvi
Caenorhabditis elegans somatic protein homeostasis (proteostasis) is actively remodeled at the onset of reproduction. This proteostatic collapse is regulated cell-nonautonomously by signals from the reproductive system that transmit the commitment to reproduction to somatic cells. Here, we asked whether the link between the reproductive system and somatic proteostasis could be uncoupled by activating downstream effectors in the gonadal longevity cascade. Specifically, we examined whether over-expression of lipl-4 (lipl-4(oe)), a target gene of the gonadal longevity pathway, or increase in arachidonic acid (AA) levels, associated with lipl-4(oe), modulated proteostasis and reproduction...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28502807/augmenting-brain-metabolism-to-increase-macro-and-chaperone-mediated-autophagy-for-decreasing-neuronal-proteotoxicity-and-aging
#10
REVIEW
Ben Loos, Daniel J Klionsky, Esther Wong
Accumulation of toxic protein aggregates in the nerve cells is a hallmark of neuronal diseases and brain aging. Mechanisms to enhance neuronal surveillance to improve neuronal proteostasis have a direct impact on promoting neuronal health and forestalling age-related decline in brain function. Autophagy is a lysosomal degradative pathway pivotal for neuronal protein quality control. Different types of autophagic mechanisms participate in protein handling in neurons. Macroautophagy targets misfolded and aggregated proteins in autophagic vesicles to the lysosomes for destruction, while chaperone-mediated autophagy (CMA) degrades specific soluble cytosolic proteins delivered to the lysosomes by chaperones...
May 11, 2017: Progress in Neurobiology
https://www.readbyqxmd.com/read/28498720/protein-misfolding-amyloid-formation-and-human-disease-a-summary-of-progress-over-the-last-decade
#11
Fabrizio Chiti, Christopher M Dobson
Peptides and proteins have been found to possess an inherent tendency to convert from their native functional states into intractable amyloid aggregates. This phenomenon is associated with a range of increasingly common human disorders, including Alzheimer and Parkinson diseases, type II diabetes, and a number of systemic amyloidoses. In this review, we describe this field of science with particular reference to the advances that have been made over the last decade in our understanding of its fundamental nature and consequences...
May 12, 2017: Annual Review of Biochemistry
https://www.readbyqxmd.com/read/28495501/semen-modulated-secretory-activity-of-oviductal-epithelial-cells-is-linked-to-cellular-proteostasis-network-remodeling-proteomic-insights-into-the-early-phase-of-interaction-in-the-oviduct-in-vivo
#12
Birgit Steinberger, Hans Yu, Theodor Brodmann, Daniela Milovanovic, Ursula Reichart, Urban Besenfelder, Konstantin Artemenko, Ebrahim Razzazi-Fazeli, Gottfried Brem, Corina Mayrhofer
The oviductal epithelium is crucial for the integrity of the female organ. Previously we got evidence that the surface proteome of oviductal epithelial cells (Oecs) is promptly altered in response to insemination and thus suggested that this early phase plays a notable regulatory role in maintaining cellular function. This study further aimed to assess the effect of semen on the cellular and molecular mechanisms in rabbit Oecs. A quantitative gel-based proteomic approach was applied to analyze changes at three time points (0h, 1h, 2h) after intrauterine insemination (IUI) compared to time matched controls...
May 8, 2017: Journal of Proteomics
https://www.readbyqxmd.com/read/28493471/what-role-does-the-stress-response-have-in-congestive-heart-failure
#13
REVIEW
Ahmed Badreddin, Youssef Fady, Hamdy Attia, Mohamed Hafez, Ahmed Khairallah, Dina Johar, Larry Bernstein
This review is concerned with cardiac malfunction as a result of an imbalance in protein proteostasis, the homeostatic balance between protein removal and regeneration in a long remodeling process involving the endoplasmic reticulum (ER) and the unfolded protein response (UPR). The importance of this is of special significance with regard to cardiac function as a high energy requiring muscular organ that has a high oxygen requirement and is highly dependent on mitochondria. The importance of mitochondria is not only concerned with high energy dependence on mitochondrial electron transport, but it also has a role in the signaling between the mitochondria and the ER under stress...
May 11, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28489421/mechanisms-and-functions-of-spatial-protein-quality-control
#14
Emily Mitchell Sontag, Rahul S Samant, Judith Frydman
A healthy proteome is essential for cell survival. Protein misfolding is linked to a rapidly expanding list of human diseases, ranging from neurodegenerative diseases to aging and cancer. Many of these diseases are characterized by the accumulation of misfolded proteins in intra- and extracellular inclusions, such as amyloid plaques. The clear link between protein misfolding and disease highlights the need to better understand the elaborate machinery that manages proteome homeostasis, or proteostasis, in the cell...
May 10, 2017: Annual Review of Biochemistry
https://www.readbyqxmd.com/read/28487627/endoplasmic-reticulum-malfunction-in-the-nervous-system
#15
Joanna Jung, Marek Michalak, Luis B Agellon
Neurodegenerative diseases often have multifactorial causes and are progressive diseases. Some are inherited while others are acquired, and both vary greatly in onset and severity. Impaired endoplasmic reticulum (ER) proteostasis, involving Ca(2+) signaling, protein synthesis, processing, trafficking, and degradation, is now recognized as a key risk factor in the pathogenesis of neurological disorders. Lipidostasis involves lipid synthesis, quality control, membrane assembly as well as sequestration of excess lipids or degradation of damaged lipids...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28487364/specific-sequences-in-the-n-terminal-domain-of-human-small-heat-shock-protein-hspb6-dictate-preferential-heterooligomerization-with-the-orthologue-hspb1
#16
Michelle Heirbaut, Frederik Lermyte, Esther M Martin, Steven Beelen, Frank Sobott, Sergei V Strelkov, Stephen D Weeks
Small heat shock proteins (sHSPs) are a conserved group of molecular chaperones with important roles in cellular proteostasis. Although sHSPs are characterized by their small monomeric weight, they typically assemble into large polydisperse oligomers that vary in both size and shape but are principally composed of dimeric building blocks. These assemblies can comprise different sHSP orthologues, creating additional complexity that may affect chaperone activity. However, the structural and functional properties of such heterooligomers are poorly understood...
May 9, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28484965/the-protective-role-of-small-heat-shock-proteins-in-cardiac-diseases-key-role-in-atrial-fibrillation
#17
Xu Hu, Denise M S Van Marion, Marit Wiersma, Deli Zhang, Bianca J J M Brundel
Atrial fibrillation (AF) is the most common tachyarrhythmia which is associated with increased morbidity and mortality. AF usually progresses from a self-terminating paroxysmal to persistent disease. It has been recognized that AF progression is driven by structural remodeling of cardiomyocytes, which results in electrical and contractile dysfunction of the atria. We recently showed that structural remodeling is rooted in derailment of proteostasis, i.e., homeostasis of protein production, function, and degradation...
May 8, 2017: Cell Stress & Chaperones
https://www.readbyqxmd.com/read/28484090/inhibition-of-stress-inducible-hsp70-impairs-mitochondrial-proteostasis-and-function
#18
Julia I-Ju Leu, Thibaut Barnoud, Gao Zhang, Tian Tian, Zhi Wei, Meenhard Herlyn, Maureen E Murphy, Donna L George
Protein quality control is an important component of survival for all cells. The use of proteasome inhibitors for cancer therapy derives from the fact that tumor cells generally exhibit greater levels of proteotoxic stress than do normal cells, and thus cancer cells tend to be more sensitive to proteasome inhibition. However, this approach has been limited in some cases by toxicity to normal cells. Recently, the concept of inhibiting proteostasis in organelles for cancer therapy has been advanced, in part because it is predicted to have reduced toxicity for normal cells...
April 21, 2017: Oncotarget
https://www.readbyqxmd.com/read/28475885/proteostatic-and-metabolic-control-of-stemness
#19
REVIEW
Laura García-Prat, Pedro Sousa-Victor, Pura Muñoz-Cánoves
Adult stem cells, particularly those resident in tissues with little turnover, are largely quiescent and only activate in response to regenerative demands, while embryonic stem cells continuously replicate, suggesting profoundly different regulatory mechanisms within distinct stem cell types. In recent years, evidence linking metabolism, mitochondrial dynamics, and protein homeostasis (proteostasis) as fundamental regulators of stem cell function has emerged. Here, we discuss new insights into how these networks control potency, self-renewal, differentiation, and aging of highly proliferative embryonic stem cells and quiescent adult stem cells, with a focus on hematopoietic and muscle stem cells and implications for anti-aging research...
May 4, 2017: Cell Stem Cell
https://www.readbyqxmd.com/read/28475037/senp8-limits-aberrant-neddylation-of-nedd8-pathway-components-to-promote-cullin-ring-ubiquitin-ligase-function
#20
Kate E Coleman, Miklós Békés, Jessica R Chapman, Sarah B Crist, Mathew Jk Jones, Beatrix M Ueberheide, Tony T Huang
NEDD8 is a ubiquitin-like modifier most well-studied for its role in activating the largest family of ubiquitin E3 ligases, the cullin-RING ligases (CRLs). While many non-cullin neddylation substrates have been proposed over the years, validation of true NEDD8 targets has been challenging, as overexpression of exogenous NEDD8 can trigger NEDD8 conjugation through the ubiquitylation machinery. Here, we developed a deconjugation-resistant form of NEDD8 to stabilize the neddylated form of cullins and other non-cullin substrates...
May 5, 2017: ELife
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