Search Results - "Shneider, A."
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Feeding ecology of juvenile dog snapper Lutjanus jocu (Bloch and Shneider, 1801) (Lutjanidae) in intertidal mangrove creeks in Curuçá estuary (Northern Brazil)
Published in Brazilian archives of biology and technology (01-12-2009)“…The diet and feeding ecology of juvenile dog snapper (Lutjanus jocu) were investigated in 92 specimens collected in four intertidal mangrove creeks of Curuçá…”
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Antisense oligonucleotide silencing of FUS expression as a therapeutic approach in amyotrophic lateral sclerosis
Published in Nature medicine (01-01-2022)“…Fused in sarcoma (FUS) is an RNA-binding protein that is genetically and pathologically associated with rare and aggressive forms of amyotrophic lateral…”
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FUS affects circular RNA expression in murine embryonic stem cell-derived motor neurons
Published in Nature communications (30-03-2017)“…The RNA-binding protein FUS participates in several RNA biosynthetic processes and has been linked to the pathogenesis of amyotrophic lateral sclerosis (ALS)…”
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ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function
Published in Nature communications (04-02-2016)“…Mutations in FUS cause amyotrophic lateral sclerosis (ALS), including some of the most aggressive, juvenile-onset forms of the disease. FUS loss-of-function…”
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Gamma motor neurons survive and exacerbate alpha motor neuron degeneration in ALS
Published in Proceedings of the National Academy of Sciences - PNAS (20-12-2016)“…The molecular and cellular basis of selective motor neuron (MN) vulnerability in amyotrophic lateral sclerosis (ALS) is not known. In genetically distinct…”
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Antisense Proline-Arginine RAN Dipeptides Linked to C9ORF72-ALS/FTD Form Toxic Nuclear Aggregates that Initiate In Vitro and In Vivo Neuronal Death
Published in Neuron (Cambridge, Mass.) (17-12-2014)“…Expanded GGGGCC (G4C2) nucleotide repeats within the C9ORF72 gene are the most common genetic mutation associated with both amyotrophic lateral sclerosis (ALS)…”
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Mutant TDP-43 Causes Early-Stage Dose-Dependent Motor Neuron Degeneration in a TARDBP Knockin Mouse Model of ALS
Published in Cell reports (Cambridge) (08-01-2019)“…Rare mutations in TARDBP, the gene encoding TDP-43, cause amyotrophic lateral sclerosis (ALS), and TDP-43 pathology is seen in a large majority of ALS…”
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The C9ORF72 GGGGCC expansion forms RNA G-quadruplex inclusions and sequesters hnRNP H to disrupt splicing in ALS brains
Published in eLife (13-09-2016)“…An expanded GGGGCC hexanucleotide in (C9) is the most frequent known cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). It has…”
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ALS/FTD Mutation-Induced Phase Transition of FUS Liquid Droplets and Reversible Hydrogels into Irreversible Hydrogels Impairs RNP Granule Function
Published in Neuron (Cambridge, Mass.) (18-11-2015)“…The mechanisms by which mutations in FUS and other RNA binding proteins cause ALS and FTD remain controversial. We propose a model in which low-complexity (LC)…”
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The ALS-associated proteins FUS and TDP-43 function together to affect Drosophila locomotion and life span
Published in The Journal of clinical investigation (01-10-2011)“…The fatal adult motor neuron disease amyotrophic lateral sclerosis (ALS) shares some clinical and pathological overlap with frontotemporal dementia (FTD), an…”
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Acute liver failure in children: They really are not just small adults: Acute liver failure in children: The first 348 patients in the Pediatric Acute Liver Failure Study Group. Squires RH Jr, Shneider BL, Bucuvalas J, Alonso E, Sokol RJ, Narkewicz, et al. J Pediatr 2006;148:652‐658
Published in Liver transplantation (01-11-2006)“…Objectives To determine short‐term outcome for children with acute liver failure (ALF) as it relates to cause, clinical status, and patient demographics and to…”
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Hypoexcitability precedes denervation in the large fast-contracting motor units in two unrelated mouse models of ALS
Published in eLife (27-03-2018)“…Hyperexcitability has been suggested to contribute to motoneuron degeneration in amyotrophic lateral sclerosis (ALS). If this is so, and given that the…”
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FUS-SMN Protein Interactions Link the Motor Neuron Diseases ALS and SMA
Published in Cell reports (Cambridge) (25-10-2012)“…Mutations in the RNA binding protein FUS cause amyotrophic lateral sclerosis (ALS), a fatal adult motor neuron disease. Decreased expression of SMN causes the…”
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Amyotrophic Lateral Sclerosis
Published in The New England journal of medicine (31-05-2001)“…Charcot described amyotrophic lateral sclerosis (ALS) in 1874. Despite progress, this creeping paralysis, known colloquially as Lou Gehrig's disease, is still…”
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Monitoring peripheral nerve degeneration in ALS by label-free stimulated Raman scattering imaging
Published in Nature communications (31-10-2016)“…The study of amyotrophic lateral sclerosis (ALS) and potential interventions would be facilitated if motor axon degeneration could be more readily visualized…”
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A Regulatory Circuitry Between Gria2, miR-409, and miR-495 Is Affected by ALS FUS Mutation in ESC-Derived Motor Neurons
Published in Molecular neurobiology (01-10-2018)“…Mutations in fused in sarcoma (FUS) cause amyotrophic lateral sclerosis (ALS). FUS is a multifunctional protein involved in the biogenesis and activity of…”
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Unexpected similarities between C9ORF72 and sporadic forms of ALS/FTD suggest a common disease mechanism
Published in eLife (13-07-2018)“…Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) represent two ends of a disease spectrum with shared clinical, genetic and pathological…”
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A new approach for rare variation collapsing on functional protein domains implicates specific genic regions in ALS
Published in Genome research (01-05-2019)“…Large-scale sequencing efforts in amyotrophic lateral sclerosis (ALS) have implicated novel genes using gene-based collapsing methods. However, pathogenic…”
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Development of a Cell Culture System From Gill Explants of the Grouper, Epinephelus malabaricus (Bloch and Shneider)
Published in Asian fisheries science (30-09-2009)Get full text
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