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Zebrafish neurodegeneration

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https://www.readbyqxmd.com/read/30454072/stable-transgenic-c9orf72-zebrafish-model-key-aspects-of-the-als-ftd-phenotype-and-reveal-novel-pathological-features
#1
Matthew P Shaw, Adrian Higginbottom, Alexander McGown, Lydia M Castelli, Evlyn James, Guillaume M Hautbergue, Pamela J Shaw, Tennore M Ramesh
A hexanucleotide repeat expansion (HRE) within the chromosome 9 open reading frame 72 (C9orf72) gene is the most prevalent cause of amyotrophic lateral sclerosis/fronto-temporal dementia (ALS/FTD). Current evidence suggests HREs induce neurodegeneration through accumulation of RNA foci and/or dipeptide repeat proteins (DPR). C9orf72 patients are known to have transactive response DNA binding protein 43 kDa (TDP-43) proteinopathy, but whether there is further cross over between C9orf72 pathology and the pathology of other ALS sub-types has yet to be revealed...
November 19, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/30424008/integrated-proteomics-and-lipidomics-investigation-of-the-mechanism-underlying-the-neuroprotective-effect-of-n-benzylhexadecanamide
#2
Yanyan Zhou, Hongjie Wang, Feifei Guo, Nan Si, Adelheid Brantner, Jian Yang, Lingyu Han, Xiaolu Wei, Haiyu Zhao, Baolin Bian
Macamides are very important secondary metabolites produced by Lepidium meyenii Walp, which possess multiple bioactivities, especially in the neuronal system. In a previous study, we observed that macamides exhibited excellent effects in the recovery of injured nerves after 1-methyl-4-phenylpyridinium (MPP⁺)-induced dopaminergic neuronal damage in zebrafish. However, the mechanism underlying this effect remains unclear. In the present study, we observed that N -benzylhexadecanamide (XA), which is a typical constituent of macamides, improved the survival rate of neurons in vitro...
November 9, 2018: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
https://www.readbyqxmd.com/read/30302279/zebrafish-an-emerging-real-time-model-system-to-study-alzheimer-s-disease-and-neurospecific-drug-discovery
#3
Suraiya Saleem, Rajaretinam Rajesh Kannan
Zebrafish ( Danio rerio ) is emerging as an increasingly successful model for translational research on human neurological disorders. In this review, we appraise the high degree of neurological and behavioural resemblance of zebrafish with humans. It is highly validated as a powerful vertebrate model for investigating human neurodegenerative diseases. The neuroanatomic and neurochemical pathways of zebrafish brain exhibit a profound resemblance with the human brain. Physiological, emotional and social behavioural pattern similarities between them have also been well established...
2018: Cell Death Discovery
https://www.readbyqxmd.com/read/30221726/silencing-of-pantothenate-kinase-2-reduces-endothelial-cell-angiogenesis
#4
Francesca Pagani, Akansha Trivedi, Deepak Khatri, Daniela Zizioli, Emirena Garrafa, Stefania Mitola, Dario Finazzi
Coenzyme A (CoA) is an essential cofactor of cellular metabolism that is involved in ~4% of cellular reactions. Its de novo production relies on five subsequent enzymatic steps, starting with the phosphorylation of vitamin B5. Pantothenate kinase 2 (PANK2) and coenzyme A synthase (COASY) catalyze the first and last steps of this pathway. Mutations in these genes lead to severe and progressive movement disorders, with neurodegeneration and iron accumulation in the basal ganglia, known as PANK2‑ and COASY protein‑associated neurodegeneration, respectively...
September 13, 2018: Molecular Medicine Reports
https://www.readbyqxmd.com/read/30193184/real-time-visualization-of-oxidative-stress-mediated-neurodegeneration-of-individual-spinal-motor-neurons-in-vivo
#5
Isabel Formella, Adam J Svahn, Rowan A W Radford, Emily K Don, Nicholas J Cole, Alison Hogan, Albert Lee, Roger S Chung, Marco Morsch
Generation of reactive oxygen species (ROS) has been shown to be important for many physiological processes, ranging from cell differentiation to apoptosis. With the development of the genetically encoded photosensitiser KillerRed (KR) it is now possible to efficiently produce ROS dose-dependently in a specific cell type upon green light illumination. Zebrafish are the ideal vertebrate animal model for these optogenetic methods because of their transparency and efficient transgenesis. Here we describe a zebrafish model that expresses membrane-targeted KR selectively in motor neurons...
October 2018: Redox Biology
https://www.readbyqxmd.com/read/30141000/overexpression-of-human-mutant-pank2-proteins-affects-development-and-motor-behavior-of-zebrafish-embryos
#6
D Khatri, D Zizioli, A Trivedi, G Borsani, E Monti, D Finazzi
Pantothenate Kinase-Associated Neurodegeneration (PKAN) is a genetic and early-onset neurodegenerative disorder characterized by iron accumulation in the basal ganglia. It is due to mutations in Pantothenate Kinase 2 (PANK2), an enzyme that catalyzes the phosphorylation of vitamin B5, first and essential step in coenzyme A (CoA) biosynthesis. Most likely, an unbalance of the neuronal levels of this important cofactor represents the initial trigger of the neurodegenerative process, yet a complete understanding of the connection between PANK2 malfunctioning and neuronal death is lacking...
August 23, 2018: Neuromolecular Medicine
https://www.readbyqxmd.com/read/29959261/biallelic-sqstm1-mutations-in-early-onset-variably-progressive-neurodegeneration
#7
Valentina Muto, Elisabetta Flex, Zachary Kupchinsky, Guido Primiano, Hamid Galehdari, Mohammadreza Dehghani, Serena Cecchetti, Giovanna Carpentieri, Teresa Rizza, Neda Mazaheri, Alireza Sedaghat, Mohammad Yahya Vahidi Mehrjardi, Alice Traversa, Michela Di Nottia, Maria M Kousi, Yalda Jamshidi, Andrea Ciolfi, Viviana Caputo, Reza Azizi Malamiri, Francesca Pantaleoni, Simone Martinelli, Aaron R Jeffries, Jawaher Zeighami, Amir Sherafat, Daniela Di Giuda, Gholam Reza Shariati, Rosalba Carrozzo, Nicholas Katsanis, Reza Maroofian, Serenella Servidei, Marco Tartaglia
OBJECTIVE: To characterize clinically and molecularly an early-onset, variably progressive neurodegenerative disorder characterized by a cerebellar syndrome with severe ataxia, gaze palsy, dyskinesia, dystonia, and cognitive decline affecting 11 individuals from 3 consanguineous families. METHODS: We used whole-exome sequencing (WES) (families 1 and 2) and a combined approach based on homozygosity mapping and WES (family 3). We performed in vitro studies to explore the effect of the nontruncating SQSTM1 mutation on protein function and the effect of impaired SQSTM1 function on autophagy...
July 24, 2018: Neurology
https://www.readbyqxmd.com/read/29949766/inhibition-of-lysosome-membrane-recycling-causes-accumulation-of-gangliosides-that-contribute-to-neurodegeneration
#8
Maxime Boutry, Julien Branchu, Céline Lustremant, Claire Pujol, Julie Pernelle, Raphaël Matusiak, Alexandre Seyer, Marion Poirel, Emeline Chu-Van, Alexandre Pierga, Kostantin Dobrenis, Jean-Philippe Puech, Catherine Caillaud, Alexandra Durr, Alexis Brice, Benoit Colsch, Fanny Mochel, Khalid Hamid El Hachimi, Giovanni Stevanin, Frédéric Darios
Lysosome membrane recycling occurs at the end of the autophagic pathway and requires proteins that are mostly encoded by genes mutated in neurodegenerative diseases. However, its implication in neuronal death is still unclear. Here, we show that spatacsin, which is required for lysosome recycling and whose loss of function leads to hereditary spastic paraplegia 11 (SPG11), promotes clearance of gangliosides from lysosomes in mouse and human SPG11 models. We demonstrate that spatacsin acts downstream of clathrin and recruits dynamin to allow lysosome membrane recycling and clearance of gangliosides from lysosomes...
June 26, 2018: Cell Reports
https://www.readbyqxmd.com/read/29938129/mechanisms-of-pathology-induced-neural-stem-cell-plasticity-and-neural-regeneration-in-adult-zebrafish-brain
#9
REVIEW
Caghan Kizil
Purpose of the Review: The purpose of this study is to review the current knowledge on the damage-induced molecular programs that underlie the regenerative ability in zebrafish brain. Recent Findings: Neural stem cells are the reservoir for new neurons during development and regeneration of the vertebrate brains. Pathological conditions such as neurodegenerative diseases hamper neural stem cell plasticity and neurogenic outcome in humans, whereas adult zebrafish brain can enhance proliferation and neurogenic capacity of its neural stem cells despite the incipient pathology...
2018: Current Pathobiology Reports
https://www.readbyqxmd.com/read/29901635/rapamycin-treatment-for-amyotrophic-lateral-sclerosis-protocol-for-a-phase-ii-randomized-double-blind-placebo-controlled-multicenter-clinical-trial-rap-als-trial
#10
RANDOMIZED CONTROLLED TRIAL
Jessica Mandrioli, Roberto D'Amico, Elisabetta Zucchi, Annalisa Gessani, Nicola Fini, Antonio Fasano, Claudia Caponnetto, Adriano Chiò, Eleonora Dalla Bella, Christian Lunetta, Letizia Mazzini, Kalliopi Marinou, Gianni Sorarù, Sara de Biasi, Domenico Lo Tartaro, Marcello Pinti, Andrea Cossarizza
INTRODUCTION: Misfolded aggregated proteins and neuroinflammation significantly contribute to amyotrophic lateral sclerosis (ALS) pathogenesis, hence representing therapeutic targets to modify disease expression. Rapamycin inhibits mechanistic target of Rapamycin (mTOR) pathway and enhances autophagy with demonstrated beneficial effects in neurodegeneration in cell line and animal models, improving phenotype in SQSTM1 zebrafish, in Drosophila model of ALS-TDP, and in the TDP43 mouse model, in which it reduced neuronal loss and TDP43 inclusions...
June 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29752287/-rnaset2-knockout-rats-exhibit-hippocampal-neuropathology-and-deficits-in-memory
#11
Kerstin W Sinkevicius, Thomas R Morrison, Praveen Kulkarni, Martha K Caffrey Cagliostro, Sade Iriah, Samantha Malmberg, Julia Sabrick, Jennifer A Honeycutt, Kim L Askew, Malav Trivedi, Craig F Ferris
RNASET2 deficiency in humans is associated with infant cystic leukoencephalopathy, which causes psychomotor impairment, spasticity and epilepsy. A zebrafish mutant model suggests that loss of RNASET2 function leads to neurodegeneration due to the accumulation of non-degraded RNA in the lysosomes. The goal of this study was to characterize the first rodent model of RNASET2 deficiency. The brains of 3- and 12-month-old RNaseT2 knockout rats were studied using multiple magnetic resonance imaging modalities and behavioral tests...
June 27, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29522758/c9orf72-is-essential-for-neurodevelopment-and-motility-mediated-by-cyclin-g1
#12
Tu-Hsueh Yeh, Han-Fang Liu, Yu-Wen Li, Chin-Song Lu, Hung-Yu Shih, Ching-Chi Chiu, Sheng-Jia Lin, Yin-Cheng Huang, Yi-Chuan Cheng
Hexanucleotide repeat expansions in the C9orf72 gene are a common genetic cause of familial and sporadic amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). However, the function of C9orf72 in neural development and the pathogenic mechanism underlying neurodegeneration are unknown. We found that disrupting C9orf72 expression by using C9orf72 constructs that lack the complete DENN domain result in reduced GTPase activity in zebrafish embryos, demonstrating the indispensability of the complete DENN domain...
June 2018: Experimental Neurology
https://www.readbyqxmd.com/read/29507920/multimodal-la-icp-ms-and-nanosims-imaging-enables-copper-mapping-within-photoreceptor-megamitochondria-in-a-zebrafish-model-of-menkes-disease
#13
Cheri M Ackerman, Peter K Weber, Tong Xiao, Bao Thai, Tiffani J Kuo, Emily Zhang, Jennifer Pett-Ridge, Christopher J Chang
Copper is essential for eukaryotic life, and animals must acquire this nutrient through the diet and distribute it to cells and organelles for proper function of biological targets. Indeed, mutations in the central copper exporter ATP7A contribute to a spectrum of diseases, including Menkes disease, with symptoms ranging from neurodegeneration to lax connective tissue. As such, a better understanding of the fundamental impacts of ATP7A mutations on in vivo copper distributions is of relevance to those affected by these diseases...
March 1, 2018: Metallomics: Integrated Biometal Science
https://www.readbyqxmd.com/read/29475037/lanthionine-ketimine-5-ethyl-ester-provides-neuroprotection-in-a-zebrafish-model-of-okadaic-acid-induced-alzheimer-s-disease
#14
Daniel Koehler, Zahoor A Shah, Kenneth Hensley, Frederick E Williams
Okadaic acid (OKA) is a protein phosphatase 2A inhibitor that is used to induce neurodegeneration and study disease states such as Alzheimer's disease (AD). Lanthionine ketimine-5-ethyl ester (LKE) is a bioavailable derivative of the naturally occurring brain sulfur metabolite, lanthionine ketimine (LK). In previously conducted studies, LKE exhibited neuroprotective and neurotrophic properties in murine models but its mechanism of action remains to be clarified. In this study, a recently established zebrafish OKA-induced AD model was utilized to further elucidate the neuroprotective and neurotrophic properties of LKE in the context of an AD-like condition...
May 2018: Neurochemistry International
https://www.readbyqxmd.com/read/29423877/nhlrc2-variants-identified-in-patients-with-fibrosis-neurodegeneration-and-cerebral-angiomatosis-finca-characterisation-of-a-novel-cerebropulmonary-disease
#15
Johanna Uusimaa, Riitta Kaarteenaho, Teija Paakkola, Hannu Tuominen, Minna K Karjalainen, Javad Nadaf, Teppo Varilo, Meri Uusi-Mäkelä, Maria Suo-Palosaari, Ilkka Pietilä, Anniina E Hiltunen, Lloyd Ruddock, Heli Alanen, Ekaterina Biterova, Ilkka Miinalainen, Annamari Salminen, Raija Soininen, Aki Manninen, Raija Sormunen, Mika Kaakinen, Reetta Vuolteenaho, Riitta Herva, Päivi Vieira, Teija Dunder, Hannaleena Kokkonen, Jukka S Moilanen, Heikki Rantala, Lawrence M Nogee, Jacek Majewski, Mika Rämet, Mikko Hallman, Reetta Hinttala
A novel multi-organ disease that is fatal in early childhood was identified in three patients from two non-consanguineous families. These children were born asymptomatic but at the age of 2 months they manifested progressive multi-organ symptoms resembling no previously known disease. The main clinical features included progressive cerebropulmonary symptoms, malabsorption, progressive growth failure, recurrent infections, chronic haemolytic anaemia and transient liver dysfunction. In the affected children, neuropathology revealed increased angiomatosis-like leptomeningeal, cortical and superficial white matter vascularisation and congestion, vacuolar degeneration and myelin loss in white matter, as well as neuronal degeneration...
May 2018: Acta Neuropathologica
https://www.readbyqxmd.com/read/29415125/elongator-subunit-3-elp3-modifies-als-through-trna-modification
#16
Andre Bento-Abreu, Gunilla Jager, Bart Swinnen, Laura Rué, Stijn Hendrickx, Ashley Jones, Kim A Staats, Ines Taes, Caroline Eykens, Annelies Nonneman, Rik Nuyts, Mieke Timmers, Lara Silva, Alain Chariot, Laurent Nguyen, John Ravits, Robin Lemmens, Deirdre Cabooter, Ludo Van Den Bosch, Philip Van Damme, Ammar Al-Chalabi, Anders Bystrom, Wim Robberecht
Amyotrophic lateral sclerosis (ALS) is a fatal degenerative motor neuron disorder of which the progression is influenced by several disease-modifying factors. Here, we investigated ELP3, a subunit of the elongator complex that modifies tRNA wobble uridines, as one of such ALS disease modifiers. ELP3 attenuated the axonopathy of a mutant SOD1, as well as of a mutant C9orf72 ALS zebrafish model. Furthermore, the expression of ELP3 in the SOD1G93A mouse extended the survival and attenuated the denervation in this model...
April 1, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29358166/an-ancient-conserved-role-for-prion-protein-in-learning-and-memory
#17
Patricia L A Leighton, Nathan J Nadolski, Adam Morrill, Trevor J Hamilton, W Ted Allison
The misfolding of cellular prion protein (PrPC ) to form PrP Scrapie (PrPSc ) is an exemplar of toxic gain-of-function mechanisms inducing propagated protein misfolding and progressive devastating neurodegeneration. Despite this, PrPC function in the brain is also reduced and subverted during prion disease progression; thus understanding the normal function of PrPC in healthy brains is key. Disrupting PrPC in mice has led to a myriad of controversial functions that sometimes map onto disease symptoms, including a proposed role in memory or learning...
January 22, 2018: Biology Open
https://www.readbyqxmd.com/read/29344929/pla2g6-deficiency-in-zebrafish-leads-to-dopaminergic-cell-death-axonal-degeneration-increased-%C3%AE-synuclein-expression-and-defects-in-brain-functions-and-pathways
#18
Elena Sánchez, Luis J Azcona, Coro Paisán-Ruiz
This study aimed to gain insights into the pathophysiology underlying PLA2G6-associated neurodegeneration that is implicated in three different neurological disorders, suggesting that other, unknown genetic or environmental factors might contribute to its wide phenotypic expression. To accomplish this, we downregulated the function of pla2g6 in the zebrafish nervous system, performed parkinsonism-related phenotypic characterization, and determined the effects of gene regulation upon the loss of pla2g6 function by using RNA sequencing and downstream analyses...
August 2018: Molecular Neurobiology
https://www.readbyqxmd.com/read/29313599/neurodegeneration-in-zebrafish-embryos-and-adults-after-cadmium-exposure
#19
Antonio Monaco, Teresa Capriello, Maria C Grimaldi, Valentina Schiano, Ida Ferrandino
Cadmium is a biologically non-essential metal. It is also toxic to many organs including the brain. The aim of this study was to analyse the neurodegenerative effects of this metal in embryos and adults of zebrafish exposed to sub-lethal concentrations of cadmium. The study was performed by cytochemical stainings. Six hours after fertilisation (hpf) zebrafish embryos were treated for 24 hours with 9 μM of cadmium and subsequently stained with Acridine orange in whole mount to detect apoptosis in the brain...
October 19, 2017: European Journal of Histochemistry: EJH
https://www.readbyqxmd.com/read/29230019/metabolic-profiling-of-zebrafish-danio-rerio-embryos-by-nmr-spectroscopy-reveals-multifaceted-toxicity-of-%C3%AE-methylamino-l-alanine-bmaa
#20
Upasana Roy, Laura Conklin, Jürgen Schiller, Jörg Matysik, John P Berry, A Alia
β-methylamino-L-alanine (BMAA) has been linked to several interrelated neurodegenerative diseases. Despite considerable research, specific contributions of BMAA toxicity to neurodegenerative diseases remain to be fully resolved. In the present study, we utilized state-of-the-art high-resolution magic-angle spinning nuclear magnetic resonance (HRMAS NMR), applied to intact zebrafish (Danio rerio) embryos, as a model of vertebrate development, to elucidate changes in metabolic profiles associated with BMAA exposure...
December 11, 2017: Scientific Reports
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