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https://www.readbyqxmd.com/read/29162978/exploring-the-interaction-of-drosophila-tdp-43-and-the-type-ii-voltage-gated-calcium-channel-cacophony-in-regulating-motor-function-and-behavior
#1
Kayly M Lembke, David B Morton
Amyotrophic lateral sclerosis (ALS) is the most common adult onset motor neurodegenerative disease. The cause of the disease remains obscure, and as such there is no effective treatment or cure. Amyotrophic lateral sclerosis and other neurodegenerative diseases are frequently characterized by dysfunction of the RNA-binding protein, TDP-43. Using model systems to understand the mechanisms underlying TDP-43 dysfunction should accelerate identification of therapeutic targets. A recent report has shown that motor defects caused by the deletion of the Drosophila TDP-43 ortholog, tbph, are not driven by changes in the physiology at the neuromuscular junction...
2017: Journal of Experimental Neuroscience
https://www.readbyqxmd.com/read/29159026/highlights-for-the-6th-international-ion-channel-conference-ion-channel-structure-function-disease-and-therapeutics
#2
Limei Wang, Kewei Wang
To foster communication and interactions amongst international scholars and scientists in the field of ion channel research, the 6th International Ion Channel Conference (IICC-2017) was held between June 23-27, 2017 in the eastern coastal city of Qingdao, China. The meeting consisted of 450 attendees and 130 speakers and poster presenters. The program consisted of research progress, new findings and ongoing studies that were focused on (1) Ion channel structure and function; (2) Ion channel physiology and human diseases; (3) Ion channels as targets for drug discovery; (4) Technological advances in ion channel research...
November 2017: Acta Pharmaceutica Sinica. B
https://www.readbyqxmd.com/read/29158683/animal-models-of-asthma-utility-and-limitations
#3
REVIEW
Marcelo Vivolo Aun, Rafael Bonamichi-Santos, Fernanda Magalhães Arantes-Costa, Jorge Kalil, Pedro Giavina-Bianchi
Clinical studies in asthma are not able to clear up all aspects of disease pathophysiology. Animal models have been developed to better understand these mechanisms and to evaluate both safety and efficacy of therapies before starting clinical trials. Several species of animals have been used in experimental models of asthma, such as Drosophila, rats, guinea pigs, cats, dogs, pigs, primates and equines. However, the most common species studied in the last two decades is mice, particularly BALB/c. Animal models of asthma try to mimic the pathophysiology of human disease...
2017: Journal of Asthma and Allergy
https://www.readbyqxmd.com/read/29155751/dissection-and-immunofluorescent-staining-of-mushroom-body-and-photoreceptor-neurons-in-adult-drosophila-melanogaster-brains
#4
Seth M Kelly, Alexandra Elchert, Michael Kahl
Nervous system development involves a sequential series of events that are coordinated by several signaling pathways and regulatory networks. Many of the proteins involved in these pathways are evolutionarily conserved between mammals and other eukaryotes, such as the fruit fly Drosophila melanogaster, suggesting that similar organizing principles exist during the development of these organisms. Importantly, Drosophila has been used extensively to identify cellular and molecular mechanisms regulating processes that are required in mammals including neurogenesis, differentiation, axonal guidance, and synaptogenesis...
November 6, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/29153328/tdp-43-promotes-neurodegeneration-by-impairing-chromatin-remodeling
#5
Amit Berson, Ashley Sartoris, Raffaella Nativio, Vivianna Van Deerlin, Jon B Toledo, Sílvia Porta, Shichong Liu, Chia-Yu Chung, Benjamin A Garcia, Virginia M-Y Lee, John Q Trojanowski, F Brad Johnson, Shelley L Berger, Nancy M Bonini
Regulation of chromatin structure is critical for brain development and function. However, the involvement of chromatin dynamics in neurodegeneration is less well understood. Here we find, launching from Drosophila models of amyotrophic lateral sclerosis and frontotemporal dementia, that TDP-43 impairs the induction of multiple key stress genes required to protect from disease by reducing the recruitment of the chromatin remodeler Chd1 to chromatin. Chd1 depletion robustly enhances TDP-43-mediated neurodegeneration and promotes the formation of stress granules...
November 9, 2017: Current Biology: CB
https://www.readbyqxmd.com/read/29142303/fus-toxicity-is-rescued-by-the-modulation-of-lncrna-hsr%C3%AF-expression-in-drosophila-melanogaster
#6
Luca Lo Piccolo, Salinee Jantrapirom, Yoshitaka Nagai, Masamitsu Yamaguchi
FUS is an aggregation-prone hnRNP involved in transcriptional and post-transcriptional regulation that aberrantly forms immunoreactive inclusion bodies in a range of neurological diseases classified as FUS-proteinopathies. Although FUS has been extensively examined, the underlying molecular mechanisms of these diseases have not yet been elucidated in detail. We previously reported that RNAi of the lncRNA hsrω altered the expression and sub-cellular localization of Drosophila FUS in the central nervous system of the fly...
November 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29141953/amyloid-%C3%AE-42-peptide-is-toxic-to-non-neural-cells-in-drosophila-yielding-a-characteristic-metabolite-profile-and-the-effect-can-be-suppressed-by-pi3k
#7
Mercedes Arnés, Sergio Casas-Tintó, Anders Malmendal, Alberto Ferrús
The human Aβ42 peptide is associated with Alzheimer's disease through its deleterious effects in neurons. Expressing the human peptide in adult Drosophila in a tissue- and time-controlled manner, we show that Aβ42 is also toxic in non-neural cells, neurosecretory and epithelial cell types in particular. This form of toxicity includes the aberrant signaling by Wingless morphogen leading to the eventual activation of Caspase 3. Preventing Caspase 3 activation by means of p53 keeps epithelial cells from elimination but maintains the Aβ42 toxicity yielding more severe deleterious effects to the organism...
November 15, 2017: Biology Open
https://www.readbyqxmd.com/read/29138281/a-conserved-cytoskeletal-signaling-cascade-mediates-neurotoxicity-of-ftdp-17-tau-mutations-in-vivo
#8
Farah H Bardai, Liqun Wang, Yamini Mutreja, Mythili Yenjerla, T Chris Gamblin, Mel B Feany
The microtubule binding protein tau is strongly implicated in multiple neurodegenerative disorders, including frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17), which is caused by mutations in tau. In vitro, FTDP-17 mutant versions of tau can reduce microtubule binding and increase aggregation of tau, but the mechanism by which these mutations promote disease in vivo is not clear. Here we take a combined biochemical and in vivo modeling approach to define functional properties of tau driving neurotoxicity in vivo We express wild type human tau and five FTDP-17 mutant forms of tau in Drosophila using a site-directed insertion strategy to ensure equivalent levels of expression...
November 14, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/29135937/relax-cool-down-and-scaffold-how-to-restore-surface-expression-of-folding-deficient-mutant-gpcrs-and-slc6-transporters
#9
REVIEW
H M Mazhar Asjad, Shahrooz Nasrollahi-Shirazi, Sonja Sucic, Michael Freissmuth, Christian Nanoff
Many diseases arise from mutations, which impair protein folding. The study of folding-deficient variants of G protein-coupled receptors and solute carrier 6 (SLC6) transporters has shed light on the folding trajectory, how it is monitored and how misfolding can be remedied. Reducing the temperature lowers the energy barrier between folding intermediates and thereby eliminates stalling along the folding trajectory. For obvious reasons, cooling down is not a therapeutic option. One approach to rescue misfolded variants is to use membrane-permeable orthosteric ligands...
November 14, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29128849/genetic-strategies-to-tackle-neurological-diseases-in-fruit-flies
#10
REVIEW
Mümine Şentürk, Hugo J Bellen
Drosophila melanogaster is a genetic model organism that has contributed to the discovery of numerous genes whose human homologues are associated with diseases. The development of sophisticated genetic tools to manipulate its genome accelerates the discovery of the genetic basis of undiagnosed human diseases and the elucidation of molecular pathogenic events of known and novel diseases. Here, we discuss various approaches used in flies to assess the function of the fly homologues of disease-associated genes...
November 8, 2017: Current Opinion in Neurobiology
https://www.readbyqxmd.com/read/29128351/drosophila-models-of-fop-provide-mechanistic-insight
#11
REVIEW
Viet Le, Edward Anderson, Takuya Akiyama, Kristi A Wharton
Fibrodysplasia ossificans progressiva (FOP) is a rare bone disease characterized by episodic events of heterotopic ossification (HO). All cases of FOP have been attributed to mutations in the ACVR1 gene that render the encoded BMP type I ALK2 receptor hypersensitive, resulting in the activation of BMP signaling, at inappropriate times in inappropriate locations. The episodic or sporadic nature of HO associated with FOP rests with the occurrence of specific 'triggers' that push the hypersensitive ALK2-FOP receptor into full signaling mode...
November 8, 2017: Bone
https://www.readbyqxmd.com/read/29127725/a-loss-of-function-homozygous-mutation-in-ddx59-implicates-a-conserved-dead-box-rna-helicase-in-nervous-system-development-and-function
#12
Vincenzo Salpietro, Stephanie Efthymiou, Andreea Manole, Bhawana Maurya, Sarah Wiethoff, Balasubramaniem Ashokkumar, Maria Concetta Cutrupi, Valeria Dipasquale, Sara Manti, Juan A Botia, Mina Ryten, Jana Vandrovcova, Oscar D Bello, Conceicao Bettencourt, Kshitij Mankad, Ashim Mukherjee, Mousumi Mutsuddi, Henry Houlden
We report on a homozygous frameshift deletion in DDX59 (c.185del: p.Phe62fs*13) in a family presenting with oro-facio-digital syndrome phenotype associated to a broad neurological involvement characterized by microcephaly, intellectual disability, epilepsy, and white matter signal abnormalities associated with cortical and sub-cortical ischemic events. DDX59 encodes a DEAD-box RNA helicase and its role in brain function and neurological diseases is unclear. We showed a reduction of mutant cDNA and perturbation of SHH signalling from patient-derived cell lines; furthermore, analysis of human brain gene expression provides evidence that DDX59 is enriched in oligodendrocytes and might act within pathways of leukoencephalopathies associated genes...
November 11, 2017: Human Mutation
https://www.readbyqxmd.com/read/29115989/clusterin-protects-neurons-against-intracellular-proteotoxicity
#13
Jenna M Gregory, Daniel R Whiten, Rebecca A Brown, Teresa P Barros, Janet R Kumita, Justin J Yerbury, Sandeep Satapathy, Karina McDade, Colin Smith, Leila M Luheshi, Christopher M Dobson, Mark R Wilson
It is now widely accepted in the field that the normally secreted chaperone clusterin is redirected to the cytosol during endoplasmic reticulum (ER) stress, although the physiological function(s) of this physical relocation remain unknown. We have examined in this study whether or not increased expression of clusterin is able to protect neuronal cells against intracellular protein aggregation and cytotoxicity, characteristics that are strongly implicated in a range of neurodegenerative diseases. We used the amyotrophic lateral sclerosis-associated protein TDP-43 as a primary model to investigate the effects of clusterin on protein aggregation and neurotoxicity in complementary in vitro, neuronal cell and Drosophila systems...
November 7, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29109235/glial-draper-rescues-a%C3%AE-toxicity-in-a-drosophila-model-of-alzheimer-s-disease
#14
Arpita Ray, Sean D Speese, Mary A Logan
Pathological hallmarks of Alzheimer's disease (AD) include amyloid-beta (Aβ) plaques, neurofibrillary tangles, and reactive gliosis. Glial cells offer protection against AD by engulfing extracellular Aβ peptides, but the repertoire of molecules required for glial recognition and destruction of Aβ are still unclear. Here, we show that the highly conserved glial engulfment receptor Draper/MEGF10 provides neuroprotection in an AD model of Drosophila (both sexes). Neuronal expression of human Aβ42(arc) in adult flies results in robust Aβ accumulation, neurodegeneration, locomotor dysfunction, and reduced lifespan...
November 6, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/29101004/acting-on-identity-myoblast-fusion-and-the-formation-of-the-syncytial-muscle-fiber
#15
REVIEW
Su Deng, Mafalda Azevedo, Mary Baylies
The study of Drosophila muscle development dates back to the middle of the last century. Since that time, Drosophila has proved to be an ideal system for studying muscle development, differentiation, function, and disease. As in humans, Drosophila muscle forms via a series of conserved steps, starting with muscle specification, myoblast fusion, attachment to tendon cells, interactions with motorneurons, and sarcomere and myofibril formation. The genes and mechanisms required for these processes share striking similarities to those found in humans...
October 31, 2017: Seminars in Cell & Developmental Biology
https://www.readbyqxmd.com/read/29100984/sexual-dimorphism-in-oxidant-induced-adaptive-homeostasis-in-multiple-wild-type-d-melanogaster-strains
#16
Laura C D Pomatto, Sarah Wong, John Tower, Kelvin J A Davies
Sexual dimorphism includes the physical and reproductive differences between the sexes, including differences that are conserved across species, ranging from the common fruit fly, Drosophila melanogaster, to humans. Sex-dependent variations in adaptive homeostasis, and adaptive stress responses may offer insight into the underlying mechanisms for male and female survival differences and into differences in chronic disease incidence and severity in humans. Earlier work showed sex-specific differences in adaptive responses to oxidative stressors in hybrid laboratory strains of D...
October 31, 2017: Archives of Biochemistry and Biophysics
https://www.readbyqxmd.com/read/29094110/enhanced-sleep-reverses-memory-deficits-and-underlying-pathology-in-drosophila-models-of-alzheimer-s-disease
#17
Stephane Dissel, Markus Klose, Jeff Donlea, Lijuan Cao, Denis English, Raphaelle Winsky-Sommerer, Bruno van Swinderen, Paul J Shaw
To test the hypothesis that sleep can reverse cognitive impairment during Alzheimer's disease, we enhanced sleep in flies either co-expressing human amyloid precursor protein and Beta-secretase (APP:BACE), or in flies expressing human tau. The ubiquitous expression of APP:BACE or human tau disrupted sleep. The sleep deficits could be reversed and sleep could be enhanced when flies were administered the GABA-A agonist 4,5,6,7-tetrahydroisoxazolo-[5,4-c]pyridine-3-ol (THIP). Expressing APP:BACE disrupted both Short-term memory (STM) and Long-term memory (LTM) as assessed using Aversive Phototaxic Suppression (APS) and courtship conditioning...
January 2017: Neurobiology of Sleep and Circadian Rhythms
https://www.readbyqxmd.com/read/29093681/quantitative-and-systems-pharmacology-3-network-based-identification-of-new-targets-for-natural-products-enables-potential-uses-in-aging-associated-disorders
#18
Jiansong Fang, Li Gao, Huili Ma, Qihui Wu, Tian Wu, Jun Wu, Qi Wang, Feixiong Cheng
Aging that refers the accumulation of genetic and physiology changes in cells and tissues over a lifetime has been shown a high risk of developing various complex diseases, such as neurodegenerative disease, cardiovascular disease and cancer. Over the past several decades, natural products have been demonstrated as anti-aging interveners via extending lifespan and preventing aging-associated disorders. In this study, we developed an integrated systems pharmacology infrastructure to uncover new indications for aging-associated disorders by natural products...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/29078798/proteomic-profiling-of-neuronal-mitochondria-reveals-modulators-of-synaptic-architecture
#19
Laura C Graham, Samantha L Eaton, Paula J Brunton, Abdelmadjid Atrih, Colin Smith, Douglas J Lamont, Thomas H Gillingwater, Giuseppa Pennetta, Paul Skehel, Thomas M Wishart
BACKGROUND: Neurons are highly polarized cells consisting of three distinct functional domains: the cell body (and associated dendrites), the axon and the synapse. Previously, it was believed that the clinical phenotypes of neurodegenerative diseases were caused by the loss of entire neurons, however it has recently become apparent that these neuronal sub-compartments can degenerate independently, with synapses being particularly vulnerable to a broad range of stimuli. Whilst the properties governing the differential degenerative mechanisms remain unknown, mitochondria consistently appear in the literature, suggesting these somewhat promiscuous organelles may play a role in affecting synaptic stability...
October 27, 2017: Molecular Neurodegeneration
https://www.readbyqxmd.com/read/29064844/the-smurf-transition-new-insights-on-ageing-from-end-of-life-studies-in-animal-models
#20
Michael Rera, Céline Vallot, Christel Lefrançois
PURPOSE OF REVIEW: Over the past 5 years, many articles were published concerning the prediction of high risk of mortality in apparently healthy adults, echoing the first description in 2011 of the Smurf phenotype, a harbinger of natural death in drosophila. RECENT FINDINGS: These recent findings suggest that the end-of-life is molecularly and physiologically highly stereotyped, evolutionarily conserved and predictable. SUMMARY: Taken altogether, these results from independent teams using multiple organisms including humans draw the lines of future directions in ageing research...
October 23, 2017: Current Opinion in Oncology
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