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https://www.readbyqxmd.com/read/27919782/understanding-zebrafish-cognition
#1
REVIEW
Darya A Meshalkina, Marina N Kizlyk, Elana V Kysil, Adam D Collier, David J Echevarria, Murilo S Abreu, Leonardo J G Barcellos, Cai Song, Allan V Kalueff
Zebrafish (Danio rerio) are rapidly becoming a popular model organism in translational and cognitive neuroscience research. Both larval and adult zebrafish continue to increase our understanding of cognitive mechanisms and their genetic and pharmacological modulation. Here, we discuss the developing utility of zebrafish in understanding cognitive phenotypes and their deficits, relevant to a wide range human brain disorders. We also discuss the potential of zebrafish models for high-throughput genetic mutant and small molecule screening (e...
December 2, 2016: Behavioural Processes
https://www.readbyqxmd.com/read/27898262/characterization-of-genetic-loss-of-function-of-fus-in-zebrafish
#2
Svetlana Lebedeva, António M de Jesus Domingues, Falk Butter, René F Ketting
The RNA-binding protein FUS is implicated in transcription, alternative splicing of neuronal genes and DNA repair. Mutations in FUS have been linked to human neurodegenerative diseases such as ALS (amyotrophic lateral sclerosis). We genetically disrupted fus in zebrafish (Danio rerio) using the CRISPR-Cas9 system. The fus knockout animals are fertile and did not show any distinctive phenotype. Mutation of fus induces mild changes in gene expression on the transcriptome and proteome level in the adult brain...
November 29, 2016: RNA Biology
https://www.readbyqxmd.com/read/27892483/down-regulation-of-coasy-the-gene-associated-with-nbia-vi-reduces-bmp-signaling-perturbs-dorso-ventral-patterning-and-alters-neuronal-development-in-zebrafish
#3
Deepak Khatri, Daniela Zizioli, Natascia Tiso, Nicola Facchinello, Sara Vezzoli, Alessandra Gianoncelli, Maurizio Memo, Eugenio Monti, Giuseppe Borsani, Dario Finazzi
Mutations in Pantothenate kinase 2 and Coenzyme A (CoA) synthase (COASY), genes involved in CoA biosynthesis, are associated with rare neurodegenerative disorders with brain iron accumulation. We showed that zebrafish pank2 gene plays an essential role in brain and vasculature development. Now we extended our study to coasy. The gene has high level of sequence identity with the human ortholog and is ubiquitously expressed from the earliest stages of development. The abrogation of its expression led to strong reduction of CoA content, high lethality and a phenotype resembling to that of dorsalized mutants...
November 28, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27890673/functional-validation-of-abhd12-mutations-in-the-neurodegenerative-disease-pharc
#4
Angèle Tingaud-Sequeira, Demetrio Raldúa, Julie Lavie, Guilaine Mathieu, Magali Bordier, Anja Knoll-Gellida, Pierre Rambeau, Isabelle Coupry, Michèle André, Eva Malm, Claes Möller, Sten Andreasson, Nanna D Rendtorff, Lisbeth Tranebjærg, Michel Koenig, Didier Lacombe, Cyril Goizet, Patrick J Babin
ABHD12 mutations have been linked to neurodegenerative PHARC (polyneuropathy, hearing loss, ataxia, retinitis pigmentosa, and early-onset cataract), a rare, progressive, autosomal, recessive disease. Although ABHD12 is suspected to play a role in the lysophosphatidylserine and/or endocannabinoid pathways, its precise functional role(s) leading to PHARC disease had not previously been characterized. Cell and zebrafish models were designed to demonstrate the causal link between an identified new missense mutation p...
November 23, 2016: Neurobiology of Disease
https://www.readbyqxmd.com/read/27807845/microcephaly-intractable-seizures-and-developmental-delay-caused-by-biallelic-variants-in-tbcd-further-delineation-of-a-new-chaperone-mediated-tubulinopathy
#5
Ben Pode-Shakked, Hila Barash, Limor Ziv, Karen W Gripp, Elisabetta Flex, Ortal Barel, Karen S Carvalho, Mena Scavina, Giovanni Chillemi, Marcello Niceta, Eran Eyal, Nitzan Kol, Bruria Ben-Zeev, Omer Bar-Yosef, Dina Marek-Yagel, Enrico Bertini, Angela L Duker, Yair Anikster, Marco Tartaglia, Annick Raas-Rothschild
Microtubule dynamics play a crucial role in neuronal development and function, and several neurodevelopmental disorders have been linked to mutations in genes encoding tubulins and functionally related proteins. Most recently, variants in the tubulin cofactor D (TBCD) gene, which encodes one of the five co-chaperones required for assembly and disassembly of α/β-tubulin heterodimer, were reported to underlie a recessive neurodevelopmental/neurodegenerative disorder. We report on five patients from three unrelated families, who presented with microcephaly, intellectual disability, intractable seizures, optic nerve pallor/atrophy, and cortical atrophy with delayed myelination and thinned corpus callosum on brain imaging...
November 2, 2016: Clinical Genetics
https://www.readbyqxmd.com/read/27785944/a-new-danshensu-derivative-protects-against-6-hydroxydopamine-induced-neurotoxicity-in-vitro-and-in-vivo
#6
Guozhen Cui, Luchen Shan, Yang Chen, Hefeng Zhou, Yuqiang Wang, Simon Ming-Yuen Lee
We previously reported a novel danshensu derivative ([Formula: see text])-(3,5,6-Trimethylpyrazinyl) methyl-2-acetoxy-3-(3,4-diacetoxyphenyl) propanoate (ADTM), which conferred cardioprotective and anti-thrombotic effects in vitro and in vivo. Here, we examined the neuroprotective actions of ADTM on 6-hydroxydopamine (6-OHDA)-induced neurotoxicity in PC12 cells 1 in vitro and zebrafish in vivo. Pretreatment with ADTM significantly inhibited 6-OHDA-induced cytotoxicity and production of reactive oxygen species (ROS) in PC12 cells through Akt signaling...
October 27, 2016: American Journal of Chinese Medicine
https://www.readbyqxmd.com/read/27770948/in-vitro-and-in-vivo-insulin-amyloid-degradation-mediated-by-serratiopeptidase
#7
Sanjay Kisan Metkar, Agnishwar Girigoswami, Ramachandran Murugesan, Koyeli Girigoswami
A transition of amyloidogenic protein by alternative folding pathway under certain conditions leads to the formation of protease resistant amyloid fibrils, having predominantly cross β structure. These amyloids are related to various neurodegenerative diseases and clearance of such amyloids may be a therapeutic approach for amyloid-related diseases. Insulin, that can form amyloids, is widely used as a model amyloidogenic protein for the study of various amyloid related diseases. In this study, insulin amyloids were formed in vitro and the potential of Serratiopeptidase (SP), a fibrinolytic-like serine protease, towards the dissociation of insulin amyloids was explored...
January 1, 2017: Materials Science & Engineering. C, Materials for Biological Applications
https://www.readbyqxmd.com/read/27763504/neuroprotective-effect-of-the-marine-derived-compound-11-dehydrosinulariolide-through-dj-1-related-pathway-in-in-vitro-and-in-vivo-models-of-parkinson-s-disease
#8
Chien-Wei Feng, Han-Chun Hung, Shi-Ying Huang, Chun-Hong Chen, Yun-Ru Chen, Chun-Yu Chen, San-Nan Yang, Hui-Min David Wang, Ping-Jyun Sung, Jyh-Horng Sheu, Kuan-Hao Tsui, Wu-Fu Chen, Zhi-Hong Wen
Parkinson's disease (PD) is a neurodegenerative disorder characterized by tremor, rigidity, bradykinesia, and gait impairment. In a previous study, we found that the marine-derived compound 11-dehydrosinulariolide (11-de) upregulates the Akt/PI3K pathway to protect cells against 6-hydroxydopamine (6-OHDA)-mediated damage. In the present study, SH-SY5Y, zebrafish and rats were used to examine the therapeutic effect of 11-de. The results revealed the mechanism by which 11-de exerts its therapeutic effect: the compound increases cytosolic or mitochondrial DJ-1 expression, and then activates the downstream Akt/PI3K, p-CREB, and Nrf2/HO-1 pathways...
October 17, 2016: Marine Drugs
https://www.readbyqxmd.com/read/27757989/identification-of-a-conserved-and-acute-neurodegeneration-specific-microglial-transcriptome-in-the-zebrafish
#9
Nynke Oosterhof, Inge R Holtman, Laura E Kuil, Herma C van der Linde, Erik W G M Boddeke, Bart J L Eggen, Tjakko J van Ham
Microglia are brain resident macrophages important for brain development, connectivity, homeostasis and disease. However, it is still largely unclear how microglia functions and their identity are regulated at the molecular level. Although recent transcriptomic studies have identified genes specifically expressed in microglia, the function of most of these genes in microglia is still unknown. Here, we performed RNA sequencing on microglia acutely isolated from healthy and neurodegenerative zebrafish brains...
October 19, 2016: Glia
https://www.readbyqxmd.com/read/27635438/mpep-modulates-metabotropic-glutamate-5-receptors-endogenously-expressed-in-zebrafish-brain
#10
José Luis Albasanz, Soraya Santana, Fernando Guzman-Sanchez, David Agustín León, Javier S Burgos, Mairena Martin
Due to phylogenetic proximity to the human, zebrafish has been recognized as a reliable model to study Alzheimer's disease (AD) and other central nervous system disorders. Furthermore, metabotropic glutamate receptors have been previously reported to be impaired in brain from AD patients. Metabotropic glutamate 5 (mGlu5) receptors are G-protein coupled receptors proposed as potential targets for therapy of different neurodegenerative disorders. Thus, MPEP (2-Methyl-6-(phenylethynyl)pyridine hydrochloride), a selective non-competitive mGlu5 receptors antagonist, has been suggested for pharmacological treatment of AD...
September 16, 2016: ACS Chemical Neuroscience
https://www.readbyqxmd.com/read/27631880/a-tol2-gateway-compatible-toolbox-for-the-study-of-the-nervous-system-and-neurodegenerative-disease
#11
Emily K Don, Isabel Formella, Andrew P Badrock, Thomas E Hall, Marco Morsch, Elinor Hortle, Alison Hogan, Sharron Chow, Serene S L Gwee, Jack J Stoddart, Garth Nicholson, Roger Chung, Nicholas J Cole
Currently there is a lack in fundamental understanding of disease progression of most neurodegenerative diseases, and, therefore, treatments and preventative measures are limited. Consequently, there is a great need for adaptable, yet robust model systems to both investigate elementary disease mechanisms and discover effective therapeutics. We have generated a Tol2 Gateway-compatible toolbox to study neurodegenerative disorders in zebrafish, which includes promoters for astrocytes, microglia and motor neurons, multiple fluorophores, and compatibility for the introduction of genes of interest or disease-linked genes...
September 15, 2016: Zebrafish
https://www.readbyqxmd.com/read/27543974/loss-of-function-of-slc25a46-causes-lethal-congenital-pontocerebellar-hypoplasia
#12
Jijun Wan, Janos Steffen, Michael Yourshaw, Hafsa Mamsa, Erik Andersen, Sabine Rudnik-Schöneborn, Kate Pope, Katherine B Howell, Catriona A McLean, Andrew J Kornberg, Jörg Joseph, Paul J Lockhart, Klaus Zerres, Monique M Ryan, Stanley F Nelson, Carla M Koehler, Joanna C Jen
Disturbed mitochondrial fusion and fission have been linked to various neurodegenerative disorders. In siblings from two unrelated families who died soon after birth with a profound neurodevelopmental disorder characterized by pontocerebellar hypoplasia and apnoea, we discovered a missense mutation and an exonic deletion in the SLC25A46 gene encoding a mitochondrial protein recently implicated in optic atrophy spectrum disorder. We performed functional studies that confirmed the mitochondrial localization and pro-fission properties of SLC25A46...
August 20, 2016: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/27535807/sex-specific-characterization-and-evaluation-of-the-alzheimer-s-disease-genetic-risk-factor-sorl1-in-zebrafish-during-aging-and-in-the-adult-brain-following-a-100%C3%A2-ppb-embryonic-lead-exposure
#13
Jinyoung Lee, Samuel M Peterson, Jennifer L Freeman
Developmental lead (Pb) exposure is suggested in laboratory studies to be a trigger for neurodegenerative diseases such as Alzheimer's disease (AD). Sortilin-related receptor, L (DLR class) A repeats-containing (SORL1) is a recently identified AD genetic risk factor. SORL1 has limited characterization in vertebrate models in comparison to other AD genetic risk factors. To characterize SORL1 further, protein sequence homology between humans, mice and zebrafish was analyzed and showed conservation of functional repeats and domain orientation...
August 18, 2016: Journal of Applied Toxicology: JAT
https://www.readbyqxmd.com/read/27505014/neural-stem-progenitor-cells-in-alzheimer-s-disease
#14
REVIEW
Gizem Tincer, Violeta Mashkaryan, Prabesh Bhattarai, Caghan Kizil
Alzheimer's disease (AD) is the most prevalent neurodegenerative disease and a worldwide health challenge. Different therapeutic approaches are being developed to reverse or slow the loss of affected neurons. Another plausible therapeutic way that may complement the studies is to increase the survival of existing neurons by mobilizing the existing neural stem/progenitor cells (NSPCs) - i.e. "induce their plasticity" - to regenerate lost neurons despite the existing pathology and unfavorable environment. However, there is controversy about how NSPCs are affected by the unfavorable toxic environment during AD...
March 2016: Yale Journal of Biology and Medicine
https://www.readbyqxmd.com/read/27475251/short-term-dietary-restriction-in-old-zebrafish-changes-cell-senescence-mechanisms
#15
Ayca Arslan-Ergul, Begun Erbaba, Elif Tugce Karoglu, Dilara Ozge Halim, Michelle Adams
Brain aging is marked by a decline in cognitive abilities and associated with neurodegenerative disorders. Recent studies have shown, neurogenesis continues into adulthood but is known to be decreasing during advancing age and these changes may contribute to cognitive changes. Advances, which aim to promote better aging are of paramount importance. Dietary restriction (DR) is the only non-genetic intervention that reliably extends life- and health-span. Mechanisms of how and why DR and age affect neurogenesis are not well-understood, and have not been utilized much in the zebrafish, which has become a popular model to study brain aging and neurodegenerative disease due to widely available genetic tools...
July 28, 2016: Neuroscience
https://www.readbyqxmd.com/read/27460825/znstress-a-high-throughput-drug-screening-protocol-for-identification-of-compounds-modulating-neuronal-stress-in-the-transgenic-mutant-sod1g93r-zebrafish-model-of-amyotrophic-lateral-sclerosis
#16
Alexander McGown, Dame Pamela J Shaw, Tennore Ramesh
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a lethal neurodegenerative disease with death on average within 2-3 years of symptom onset. Mutations in superoxide dismutase 1 (SOD1) have been identified to cause ALS. Riluzole, the only neuroprotective drug for ALS provides life extension of only 3 months on average. Thishighlights the need for compound screening in disease models to identify new neuroprotective therapies for this disease. Zebrafish is an emerging model system that is well suited for the study of diseasepathophysiology and also for high throughput (HT) drug screening...
2016: Molecular Neurodegeneration
https://www.readbyqxmd.com/read/27458342/function-over-form-modeling-groups-of-inherited-neurological-conditions-in-zebrafish
#17
REVIEW
Robert A Kozol, Alexander J Abrams, David M James, Elena Buglo, Qing Yan, Julia E Dallman
Zebrafish are a unique cell to behavior model for studying the basic biology of human inherited neurological conditions. Conserved vertebrate genetics and optical transparency provide in vivo access to the developing nervous system as well as high-throughput approaches for drug screens. Here we review zebrafish modeling for two broad groups of inherited conditions that each share genetic and molecular pathways and overlap phenotypically: neurodevelopmental disorders such as Autism Spectrum Disorders (ASD), Intellectual Disability (ID) and Schizophrenia (SCZ), and neurodegenerative diseases, such as Cerebellar Ataxia (CATX), Hereditary Spastic Paraplegia (HSP) and Charcot-Marie Tooth Disease (CMT)...
2016: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/27425895/unconventional-neurotrophic-factors-cdnf-and-manf-structure-physiological-functions-and-therapeutic-potential
#18
Maria Lindahl, Mart Saarma, Päivi Lindholm
Cerebral dopamine neurotrophic factor (CDNF) and mesencephalic astrocyte-derived neurotrophic factor (MANF) promote the survival of midbrain dopaminergic neurons which degenerate in Parkinson's disease (PD). However, CDNF and MANF are structurally and functionally clearly distinct from the classical, target-derived neurotrophic factors (NTFs) that are solely secreted proteins. In cells, CDNF and MANF localize in the endoplasmic reticulum (ER) and evidence suggests that MANF, and possibly CDNF, is important for the maintenance of ER homeostasis...
July 14, 2016: Neurobiology of Disease
https://www.readbyqxmd.com/read/27417116/arf6-and-the-5-phosphatase-of-synaptojanin-1-regulate-autophagy-in-cone-photoreceptors
#19
Ashley A George, Sara Hayden, Gail R Stanton, Susan E Brockerhoff
Abnormalities in the ability of cells to properly degrade proteins have been identified in many neurodegenerative diseases. Recent work has implicated synaptojanin 1 (SynJ1) in Alzheimer's disease and Parkinson's disease, although the role of this polyphosphoinositide phosphatase in protein degradation has not been thoroughly described. Here, we dissected in vivo the role of SynJ1 in endolysosomal trafficking in zebrafish cone photoreceptors using a SynJ1-deficient zebrafish mutant, nrc(a14) . We found that loss of SynJ1 leads to specific accumulation of late endosomes and autophagosomes early in photoreceptor development...
July 2016: BioEssays: News and Reviews in Molecular, Cellular and Developmental Biology
https://www.readbyqxmd.com/read/27404450/quantitative-proteomics-analysis-of-zebrafish-exposed-to-sub-lethal-dosages-of-%C3%AE-methyl-amino-l-alanine-bmaa
#20
Ann Kristin Frøyset, Essa Ahsan Khan, Kari Espolin Fladmark
The non-protein amino acid β-methylamino-L-alanine (BMAA) is a neurotoxin present in microalgae and shown to accumulate in the food web. BMAA has been linked to the complex neurodegenerative disorder of Guam and to increased incidents sporadic ALS. Two main neurotoxic routes are suggested; an excitotoxic by acting as an agonist towards glutamate receptors and a metabolic by misincorporating into cellular proteins. We have used zebrafish, an increasingly used model for neurodegenerative diseases, to further identify signaling components involved in BMAA-induced toxicity...
2016: Scientific Reports
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