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Author Details

Sami J Barmada
University of Michigan ann arbor
1997
72
32
Nevan J Krogan (CM4AI)
PMIDPaper TitleJournal TitlePublished Year
36219176miRNA analysis reveals novel dysregulated pathways in amyotrophic lateral sclerosis.Hum Mol Genet2023
37986813Stress granule formation helps to mitigate neurodegeneration.bioRxiv2023
37540028The MLO-down on TDP-43.Brain2023
37011198Neuronal activity regulates Matrin 3 abundance and function in a calcium-dependent manner through calpain-mediated cleavage and calmodulin binding.Proc Natl Acad Sci U S A2023
37333274Ribosomal quality control factors inhibit repeat-associated non-AUG translation from GC-rich repeats.bioRxiv2023
37000196C-terminal frameshift variant of TDP-43 with pronounced aggregation-propensity causes rimmed vacuole myopathy but not ALS/FTD.Acta Neuropathol2023
37162852TorsinA is essential for the timing and localization of neuronal nuclear pore complex biogenesis.bioRxiv2023
36919702Glia in FTLD-GRN: from supporting cast to leading role.J Clin Invest2023
36609661Ubiquilin-2 regulates pathological alpha-synuclein.Sci Rep2023
36310225Neuronal Puncta/Aggregate Formation by WT and Mutant UBQLN2.Methods Mol Biol2023
34057020Autophagy and ALS: mechanistic insights and therapeutic implications.Autophagy2022
35581326Heat shock protein Grp78/BiP/HspA5 binds directly to TDP-43 and mitigates toxicity associated with disease pathology.Sci Rep2022
35589711Poly(GR) and poly(GA) in cerebrospinal fluid as potential biomarkers for C9ORF72-ALS/FTD.Nat Commun2022
36482422Nuclear import receptors are recruited by FG-nucleoporins to rescue hallmarks of TDP-43 proteinopathy.Mol Neurodegener2022
35904811CGG repeats trigger translational frameshifts that generate aggregation-prone chimeric proteins.Nucleic Acids Res2022
34981791Progranulin deficiency promotes neuroinflammation and neuron loss following toxin-induced injury.J Clin Invest2022
35082119Disrupting the Balance of Protein Quality Control Protein UBQLN2 Accelerates Tau Proteinopathy.J Neurosci2022
33427209Matrin 3 in neuromuscular disease: physiology and pathophysiology.JCI Insight2021
33792567ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects.J Clin Invest2021
33892814Enhanced detection of expanded repeat mRNA foci with hybridization chain reaction.Acta Neuropathol Commun2021
34303705Development of a specific live-cell assay for native autophagic flux.J Biol Chem2021
34115105TDP-43 stabilizes G3BP1 mRNA: relevance to amyotrophic lateral sclerosis/frontotemporal dementia.Brain2021
34190355A CRMP4-dependent retrograde axon-to-soma death signal in amyotrophic lateral sclerosis.EMBO J2021
32477070Cytoplasmic TDP43 Binds microRNAs: New Disease Targets in Amyotrophic Lateral Sclerosis.Front Cell Neurosci2020
31714900Shortened TDP43 isoforms upregulated by neuronal hyperactivity drive TDP43 pathology in ALS.J Clin Invest2020
32066985A native function for RAN translation and CGG repeats in regulating fragile X protein synthesis.Nat Neurosci2020
33044808An Allosteric Modulator of RNA Binding Targeting the N-Terminal Domain of TDP-43 Yields Neuroprotective Properties.ACS Chem Biol2020
32681165Ubiquilin-2 differentially regulates polyglutamine disease proteins.Hum Mol Genet2020
32763223TDP-43 Nuclear Bodies: A NEAT Response to Stress?Mol Cell2020
32753055The carboxyl termini of RAN translated GGGGCC nucleotide repeat expansions modulate toxicity in models of ALS/FTD.Acta Neuropathol Commun2020
30735193Monitoring Neuronal Survival via Longitudinal Fluorescence Microscopy.J Vis Exp2019
31649034High-throughput screening yields several small-molecule inhibitors of repeat-associated non-AUG translation.J Biol Chem2019
31347257DDX3X and specific initiation factors modulate FMR1 repeat-associated non-AUG-initiated translation.EMBO Rep2019
31018129An Intramolecular Salt Bridge Linking TDP43 RNA Binding, Protein Stability, and TDP43-Dependent Neurodegeneration.Cell Rep2019
28986324The ubiquitin conjugating enzyme Ube2W regulates solubility of the Huntington's disease protein, huntingtin.Neurobiol Dis2018
29916018RNA Degradation in Neurodegenerative Disease.Adv Neurobiol2018
30124449Gene Transfer Induced Hypercholesterolemia in Amyloid Mice.J Alzheimers Dis2018
30015619Matrin 3-dependent neurotoxicity is modified by nucleic acid binding and nucleocytoplasmic localization.Elife2018
30030424Abnormal RNA stability in amyotrophic lateral sclerosis.Nat Commun2018
30333186Mutant UBQLN2 promotes toxicity by modulating intrinsic self-assembly.Proc Natl Acad Sci U S A2018
29545601TDP43 nuclear export and neurodegeneration in models of amyotrophic lateral sclerosis and frontotemporal dementia.Sci Rep2018
29395044TDP43 and RNA instability in amyotrophic lateral sclerosis.Brain Res2018
29308690Overexpression of a conserved HSP40 chaperone reduces toxicity of several neurodegenerative disease proteins.Prion2018
28531192Overexpression of the essential Sis1 chaperone reduces TDP-43 effects on toxicity and proteolysis.PLoS Genet2017
28197542The Proline/Arginine Dipeptide from Hexanucleotide Repeat Expanded <i>C9ORF72</i> Inhibits the Proteasome.eNeuro2017
29256861Inhibition of PIP4Kγ ameliorates the pathological effects of mutant huntingtin protein.Elife2017
29222490RAN translation at C9orf72-associated repeat expansions is selectively enhanced by the integrated stress response.Nat Commun2017
27776165Distinct C9orf72-Associated Dipeptide Repeat Structures Correlate with Neuronal Toxicity.PLoS One2016
25689976Linking RNA Dysfunction and Neurodegeneration in Amyotrophic Lateral Sclerosis.Neurotherapeutics2015
26056265Amelioration of toxicity in neuronal models of amyotrophic lateral sclerosis by hUPF1.Proc Natl Acad Sci U S A2015
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J. David Gladstone Institutes
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Perelman School of Medicine at the University of Pennsylvania
Co-authored papers 6
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University of California san francisco
Co-authored papers 5
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Co-authored papers 3
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Co-authored papers 3
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University of California san francisco
Co-authored papers 2
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Gladstone Institutes
Co-authored papers 2
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College of Dentistry, New York University
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The Picower Institute for Learning and Memory, Massachusetts Institute of Technology
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Pennsylvania Muscle Institute, University of Pennsylvania Perelman School of Medicine
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Zamia Diaz
Perelman School of Medicine, University of Pennsylvania
Co-authored papers 1
Todd E Golde
Emory Center for Neurodegenerative Disease
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Leslie M Thompson
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Fen-Biao Gao
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Noel Southall
National Center for Advancing Translational Sciences, National Institutes of Health
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William P Dillon
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Mark H Ellisman
National Center for Microscopy and Imaging Research, University of California
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Juan Botas
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Release 1.2