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Protein aggregation

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https://www.readbyqxmd.com/read/29458128/aggregation-prone-regions-in-hypk-help-it-to-form-sequestration-complex-for-toxic-protein-aggregates
#1
Debasish Kumar Ghosh, Ajit Roy, Akash Ranjan
Protein aggregates result from altered structural conformations and they can perturb cellular homeostasis. Prevention mechanisms, which function against protein aggregation by modulatory processes, are diverse and redundant. In this study, we have characterized Huntingtin interacting protein K (HYPK) as a global aggregation-regulatory protein. We report the mechanistic details of how HYPK's aggregation-prone regions allow it to sense and prevent other toxic protein's aggregation by forming unique annular-shaped sequestration complexes...
February 16, 2018: Journal of Molecular Biology
https://www.readbyqxmd.com/read/29457785/tia1-variant-drives-myodegeneration-in-multisystem-proteinopathy-with-sqstm1-mutations
#2
YouJin Lee, Per Harald Jonson, Jaakko Sarparanta, Johanna Palmio, Mohona Sarkar, Anna Vihola, Anni Evilä, Tiina Suominen, Sini Penttilä, Marco Savarese, Mridul Johari, Marie-Christine Minot, David Hilton-Jones, Paul Maddison, Patrick Chinnery, Jens Reimann, Cornelia Kornblum, Torsten Kraya, Stephan Zierz, Carolyn Sue, Hans Goebel, Asim Azfer, Stuart H Ralston, Peter Hackman, Robert C Bucelli, J Paul Taylor, Conrad C Weihl, Bjarne Udd
Multisystem proteinopathy (MSP) involves disturbances of stress granule (SG) dynamics and autophagic protein degradation that underlie the pathogenesis of a spectrum of degenerative diseases that affect muscle, brain, and bone. Specifically, identical mutations in the autophagic adaptor SQSTM1 can cause varied penetrance of 4 distinct phenotypes: amyotrophic lateral sclerosis (ALS), frontotemporal dementia, Paget's disease of the bone, and distal myopathy. It has been hypothesized that clinical pleiotropy relates to additional genetic determinants, but thus far, evidence has been lacking...
February 19, 2018: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/29457782/hypothalamic-er-associated-degradation-regulates-pomc-maturation-feeding-and-age-associated-obesity
#3
Geun Hyang Kim, Guojun Shi, Diane Rm Somlo, Leena Haataja, Soobin Song, Qiaoming Long, Eduardo A Nillni, Malcolm J Low, Peter Arvan, Martin G Myers, Ling Qi
Pro-opiomelanocortin (POMC) neurons function as key regulators of metabolism and physiology by releasing prohormone-derived neuropeptides with distinct biological activities. However, our understanding of early events in prohormone maturation in the ER remains incomplete. Highlighting the significance of this gap in knowledge, a single POMC cysteine-to-phenylalanine mutation at position 28 (POMC-C28F) is defective for ER processing and causes early onset obesity in a dominant-negative manner in humans through an unclear mechanism...
February 19, 2018: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/29456521/mathematical-modeling-of-protein-misfolding-mechanisms-in-neurological-diseases-a-historical-overview
#4
REVIEW
Felix Carbonell, Yasser Iturria-Medina, Alan C Evans
Protein misfolding refers to a process where proteins become structurally abnormal and lose their specific 3-dimensional spatial configuration. The histopathological presence of misfolded protein (MP) aggregates has been associated as the primary evidence of multiple neurological diseases, including Prion diseases, Alzheimer's disease, Parkinson's disease, and Creutzfeldt-Jacob disease. However, the exact mechanisms of MP aggregation and propagation, as well as their impact in the long-term patient's clinical condition are still not well understood...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29456016/monoclonal-antibody-based-in-vitro-potency-assay-as-a-predictor-of-antigenic-integrity-and-in-vivo-immunogenicity-of-a-respiratory-syncytial-virus-post-fusion-f-protein-based-vaccine
#5
Matieyendou Didier Djagbare, Li Yu, Arun Parupudi, Jenny Sun, Melissa L Coughlin, Benjamin S Rush, Gautam Sanyal
The post-fusion form of Respiratory Syncytial Virus (RSV) fusion (F) protein has been used recently in clinical trials as a potential vaccine antigen with the objective of eliciting protective immune response against RSV. In this paper, in vitro antigenicity and in vivo immunogenicity of recombinant, soluble F protein of RSV (RSVsF) were evaluated by several assays. In Vitro Relative Potency (IVRP) of RSVsF was measured in a sandwich ELISA using two antibodies, each specific for epitope site A or C. Therefore, IVRP reflected the integrity of the antigen in terms of changes in antibody binding affinity of either or both of these sites...
February 15, 2018: Vaccine
https://www.readbyqxmd.com/read/29454758/optical-fiber-tips-for-biological-applications-from-light-confinement-biosensing-to-bioparticles-manipulation
#6
REVIEW
Joana S Paiva, Pedro A S Jorge, Carla C Rosa, João P S Cunha
BACKGROUND: The tip of an optical fiber has been considered an attractive platform in Biology. The simple cleaved end of an optical fiber can be machined, patterned and/or functionalized, acquiring unique properties enabling the exploitation of novel optical phenomena. Prompted by the constant need to measure and manipulate nanoparticles, the invention of the Scanning Near-Field Optical Microscopy (SNOM) triggered the optimization and development of novel fiber tip microfabrication methods...
February 15, 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29454054/genomic-perspectives-of-spider-silk-genes-through-target-capture-sequencing-conservation-of-stabilization-mechanisms-and-homology-based-structural-models-of-spidroin-terminal-regions
#7
Matthew A Collin, Thomas H Clarke, Nadia A Ayoub, Cheryl Y Hayashi
A powerful system for studying protein aggregation, particularly rapid self-assembly, is spider silk. Spider silks are proteinaceous and silk proteins are synthesized and stored within silk glands as liquid dope. As needed, liquid dope is near-instantaneously transformed into solid fibers or viscous adhesives. The dominant constituents of silks are spidroins (spider fibroins) and their terminal domains are vital for the tight control of silk self-assembly. To better understand spidroin termini, we used target capture and deep sequencing to identify spidroin gene sequences from six species representing the araneoid families of Araneidae, Nephilidae, and Theridiidae...
February 14, 2018: International Journal of Biological Macromolecules
https://www.readbyqxmd.com/read/29453998/ctl14-a-recognition-receptor-induced-in-late-stage-larvae-modulates-anti-fungal-immunity-in-cotton-bollworm-helicoverpa-armigera
#8
Yang Cheng, Zhe Lin, Ju-Mei Wang, Long-Sheng Xing, Guang-Hua Xiong, Zhen Zou
C-type lectin (CTL) is usually considered as pattern recognition receptors in insect innate immunity. Here we found that CTL14 of Helicoverpa armigera was only activated in the fifth instar larvae not in the second instar by entomopathogen Beauveria bassiana infection. Recombinant CTL14 protein was found to form aggregates with zymosan and B. bassiana in vitro. Immunoprecipitation studies demonstrated that CTL14 interacted with serine proteinases (SP), serine proteinase inhibitor (serpin), prophenoloxidases (PPO) and vitellogenin (Vg) in the larval hemolymph...
February 14, 2018: Developmental and Comparative Immunology
https://www.readbyqxmd.com/read/29453960/synaptic-dysfunction-and-altered-excitability-in-c9orf72-als-ftd
#9
Alexander Starr, Rita Sattler
Amyotrophic lateral sclerosis (ALS) is characterized by a progressive degeneration of upper and lower motor neurons, resulting in fatal paralysis due to denervation of the muscle. Due to genetic, pathological and symptomatic overlap, ALS is now considered a spectrum disease together with frontotemporal dementia (FTD), the second most common cause of dementia in individuals under the age of 65. Interestingly, in both diseases, there is a large prevalence of RNA binding proteins (RBPs) that are mutated and considered disease-causing, or whose dysfunction contribute to disease pathogenesis...
February 14, 2018: Brain Research
https://www.readbyqxmd.com/read/29453901/shedding-light-on-aberrant-interactions-a-review-of-modern-tools-for-studying-protein-aggregates
#10
REVIEW
Franziska Kundel, Laura Tosatto, Daniel R Whiten, David C Wirthensohn, Mathew H Horrocks, David Klenerman
The link between protein aggregation and neurodegenerative disease is well established. However, given the heterogeneity of species formed during the aggregation process, it is difficult to delineate details of the molecular events involved in generating pathological aggregates from those producing soluble monomers. As aberrant aggregates are possible pharmacological targets for the treatment of neurodegenerative diseases, the need to observe and characterise soluble oligomers has pushed traditional biophysical techniques to their limits, leading to the development of a plethora of new tools capable of detecting soluble oligomers with high precision and specificity...
February 17, 2018: FEBS Journal
https://www.readbyqxmd.com/read/29453800/atomic-structures-of-corkscrew-forming-segments-of-sod1-reveal-varied-oligomer-conformations
#11
Smriti Sangwan, Michael R Sawaya, Kevin A Murray, MichaelP Hughes, David S Eisenberg
The aggregation cascade of disease-related amyloidogenic proteins, terminating in insoluble amyloid fibrils, involves intermediate oligomeric states. The structural and biochemical details of these oligomers have been largely unknown. Here we report crystal structures of variants of the cytotoxic oligomer-forming segment residues 28-38 of the ALS-linked protein, SOD1. The crystal structures reveal three different architectures: corkscrew oligomeric structure, non-twisting curved sheet structure and a steric zipper proto-filament structure...
February 17, 2018: Protein Science: a Publication of the Protein Society
https://www.readbyqxmd.com/read/29453569/assessing-autophagy-in-sertoli-cells
#12
Chao Liu, Jehangir Khan, Wei Li
Autophagy is an important cellular homeostatic process, it degrades most long-lived proteins and some organelles by lysosome to provide raw materials for the survival of the cells during nutrient or energy deprivation condition. Autophagy is active in Sertoli cells and involved in many cellular processes. However, the precise role of autophagy in Sertoli cells is still largely unknown. Thus, the assessment of autophagy in Sertoli cells should be helpful for investigating the functional roles of autophagy in Sertoli cells...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/29453537/toxicogenomics-a-new-paradigm-for-nanotoxicity-evaluation
#13
Sourabh Dwivedi, Quaiser Saquib, Bilal Ahmad, Sabiha M Ansari, Ameer Azam, Javed Musarrat
The wider applications of nanoparticles (NPs) has evoked a world-wide concern due to their possible risk of toxicity in humans and other organisms. Aggregation and accumulation of NPs into cell leads to their interaction with biological macromolecules including proteins, nucleic acids and cellular organelles, which eventually induce toxicological effects. Application of toxicogenomics to investigate molecular pathway-based toxicological consequences has opened new vistas in nanotoxicology research. Indeed, genomic approaches appeared as a new paradigm in terms of providing information at molecular levels and have been proven to be as a powerful tool for identification and quantification of global shifts in gene expression...
2018: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29453246/yield-of-the-ryr2-genetic-test-in-suspected-catecholaminergic-polymorphic-ventricular-tachycardia-and-implications-for-test-interpretation
#14
Jamie D Kapplinger, Krishna N Pundi, Nicholas B Larson, Thomas E Callis, David J Tester, Hennie Bikker, Arthur A M Wilde, Michael J Ackerman
BACKGROUND: Pathogenic RYR2 variants account for ≈60% of clinically definite cases of catecholaminergic polymorphic ventricular tachycardia. However, the rate of rare benign RYR2 variants identified in the general population remains a challenge for genetic test interpretation. Therefore, we examined the results of the RYR2 genetic test among patients referred for commercial genetic testing and examined factors impacting variant interpretability. METHODS: Frequency and location comparisons were made for RYR2 variants identified among 1355 total patients of varying clinical certainty and 60 706 Exome Aggregation Consortium controls...
February 2018: Circ Genom Precis Med
https://www.readbyqxmd.com/read/29453052/ac-lvffark-nh-2-conjugation-to-%C3%AE-cyclodextrin-exhibits-significantly-enhanced-performance-on-inhibiting-amyloid-%C3%AE-protein-fibrillogenesis-and-cytotoxicity
#15
Huan Zhang, Xiaoyan Dong, Fufeng Liu, Jie Zheng, Yan Sun
Inhibition of amyloid β-protein (Aβ) aggregation is of significance for the potential treatment of Alzheimer's disease. We have herein conjugated heptapeptide Ac-LVFFARK-NH 2 (LK7) to β-cyclodextrin (βCyD) and studied the inhibitory effect of the LK7-βCyD conjugate on Aβ aggregation. The conjugation significantly improved the peptide solubility and suppressed the self-assembly propensity. This led to 30% increase of the binding affinity of LK7 for Aβ in the conjugate due to increased hydrophobic interactions...
February 11, 2018: Biophysical Chemistry
https://www.readbyqxmd.com/read/29450991/mass-spectrometry-based-fast-photochemical-oxidation-of-proteins-fpop-for-higher-order-structure-characterization
#16
Ke Sherry Li, Liuqing Shi, Michael L Gross
Assessment of protein structure and interaction is crucial for understanding protein structure/function relationships. Compared to high-resolution structural tools, including X-ray crystallography, nuclear magnetic resonance (NMR), and cryo-EM, and traditional low-resolution methods, such as circular dichroism, UV-vis, and florescence spectroscopy, mass spectrometry (MS)-based protein footprinting affords medium-to-high resolution (i.e., regional and residue-specific insights) by taking advantage of proteomics methods focused on the primary structure...
February 16, 2018: Accounts of Chemical Research
https://www.readbyqxmd.com/read/29449811/hdac6-inhibition-promotes-transcription-factor-eb-activation-and-is-protective-in-experimental-kidney-disease
#17
Angela S Brijmohan, Sri N Batchu, Syamantak Majumder, Tamadher A Alghamdi, Karina Thieme, Sarah McGaugh, Youan Liu, Suzanne L Advani, Bridgit B Bowskill, M Golam Kabir, Laurette Geldenhuys, Ferhan S Siddiqi, Andrew Advani
To contend with the deleterious effects of accumulating misfolded protein aggregates or damaged organelles cells rely on a system of quality control processes, among them the autophagy-lysosome pathway. This pathway is itself controlled by a master regulator transcription factor termed transcription factor EB (TFEB). When TFEB localizes to the cell nucleus it promotes the expression of a number of genes involved in protein clearance. Here, we set out to determine (1) whether TFEB expression is altered in chronic kidney disease (CKD); (2) whether inhibition of the cytosolic deacetylase histone deacetylase 6 (HDAC6) affects TFEB acetylation and nuclear localization; and (3) whether HDAC6 inhibition, in turn, alters the natural history of experimental CKD...
2018: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/29449800/robustness-and-vulnerability-of-the-autoregulatory-system-that-maintains-nuclear-tdp-43-levels-a-trade-off-hypothesis-for-als-pathology-based-on-in-silico-data
#18
Akihiro Sugai, Taisuke Kato, Akihide Koyama, Yuka Koike, Sou Kasahara, Takuya Konno, Tomohiko Ishihara, Osamu Onodera
Abnormal accumulation of TAR DNA-binding protein 43 (TDP-43) in the cytoplasm and its disappearance from the nucleus are pathological features of amyotrophic lateral sclerosis and frontotemporal dementia (ALS/FTD) and are directly involved in the pathogenesis of these conditions. TDP-43 is an essential nuclear protein that readily aggregates in a concentration-dependent manner. Therefore, cells must strictly maintain an appropriate amount of nuclear TDP-43. In one relevant maintenance mechanism, TDP-43 binds to its pre-mRNA and promotes alternative splicing, resulting in mRNA degradation via nonsense-mediated mRNA decay...
2018: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/29449775/prion-proteins-without-the-glycophosphatidylinositol-anchor-potential-biomarkers-in-neurodegenerative-diseases
#19
REVIEW
Valerija Kovač, Vladka Čurin Šerbec
Prion protein (PrP) is a biomolecule that is involved in neuronal signaling, myelinization, and the development of neurodegenerative diseases. In the cell, PrP is shed by the ADAM10 protease. This process generates PrP molecules that lack glycophosphatidylinositol anchor, and these molecules incorporate into toxic aggregates and neutralize toxic oligomers. Due to this dual role, these molecules are important biomarkers for neurodegenerative diseases. In this review, we present shed PrP as a potential biomarker, with a focus on PrP226*, which may be the main biomarker for predicting neurodegenerative diseases in humans...
2018: Biomarker Insights
https://www.readbyqxmd.com/read/29449700/imaging-tau-and-amyloid-%C3%AE-proteinopathies-in-alzheimer-disease-and-other-conditions
#20
REVIEW
Victor L Villemagne, Vincent Doré, Samantha C Burnham, Colin L Masters, Christopher C Rowe
Most neurodegenerative disorders are associated with aggregated protein deposits. In the case of Alzheimer disease (AD), extracellular amyloid-β (Aβ) aggregates and intracellular tau neurofibrillary tangles are the two neuropathological hallmarks of the disease. Aβ-PET imaging has already been approved for clinical use and is being used in clinical trials of anti-Aβ therapies both for patient recruitment and as an outcome measure. These studies have shown that Aβ accumulation is a protracted process that can extend for more than 2 decades before the onset of clinical AD...
February 16, 2018: Nature Reviews. Neurology
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