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Author Details

Guillaume M Hautbergue
Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield
1999
65
29
Nevan J Krogan (CM4AI)
PMIDPaper TitleJournal TitlePublished Year
35871544Atypical TDP-43 protein expression in an ALS pedigree carrying a p.Y374X truncation mutation in TARDBP.Brain Pathol2023
37679383The master energy homeostasis regulator PGC-1α exhibits an mRNA nuclear export function.Nat Commun2023
37251807A Y374X TDP43 truncation leads to an altered metabolic profile in amyotrophic lateral sclerosis fibroblasts driven by pyruvate and TCA cycle intermediate alterations.Front Aging Neurosci2023
36857431A cell-penetrant peptide blocking <i>C9ORF72</i>-repeat RNA nuclear export reduces the neurotoxic effects of dipeptide repeat proteins.Sci Transl Med2023
36241425Low expression of EXOSC2 protects against clinical COVID-19 and impedes SARS-CoV-2 replication.Life Sci Alliance2022
35440492SMN-deficient cells exhibit increased ribosomal DNA damage.Life Sci Alliance2022
35646086RNA Helicases in Microsatellite Repeat Expansion Disorders and Neurodegeneration.Front Genet2022
35568435<i>C9ORF72</i>-derived poly-GA DPRs undergo endocytic uptake in iAstrocytes and spread to motor neurons.Life Sci Alliance2022
36619668Loss of TMEM106B exacerbates C9ALS/FTD DPR pathology by disrupting autophagosome maturation.Front Cell Neurosci2022
33314575Directly converted astrocytes retain the ageing features of the donor fibroblasts and elucidate the astrocytic contribution to human CNS health and disease.Aging Cell2021
33729487Mechanisms of repeat-associated non-AUG translation in neurological microsatellite expansion disorders.Biochem Soc Trans2021
34376242SRSF1-dependent inhibition of C9ORF72-repeat RNA nuclear export: genome-wide mechanisms for neuroprotection in amyotrophic lateral sclerosis.Mol Neurodegener2021
34392299Therapeutic strategies for C9orf72 amyotrophic lateral sclerosis and frontotemporal dementia.Curr Opin Neurol2021
32669685Applications of machine learning to diagnosis and treatment of neurodegenerative diseases.Nat Rev Neurol2020
30698736Astrocyte adenosine deaminase loss increases motor neuron toxicity in amyotrophic lateral sclerosis.Brain2019
30811981Mutations in the Glycosyltransferase Domain of GLT8D1 Are Associated with Familial Amyotrophic Lateral Sclerosis.Cell Rep2019
30711519Micro-RNAs secreted through astrocyte-derived extracellular vesicles cause neuronal network degeneration in C9orf72 ALS.EBioMedicine2019
30267647TIGAR inclusion pathology is specific for Lewy body diseases.Brain Res2019
29738614Proteomic and cellular localisation studies suggest non-tight junction cytoplasmic and nuclear roles for occludin in astrocytes.Eur J Neurosci2018
29954071Burkholderia Lethal Factor 1, a Novel Anti-Cancer Toxin, Demonstrates Selective Cytotoxicity in MYCN-Amplified Neuroblastoma Cells.Toxins (Basel)2018
30195799Translating SOD1 Gene Silencing toward the Clinic: A Highly Efficacious, Off-Target-free, and Biomarker-Supported Strategy for fALS.Mol Ther Nucleic Acids2018
30454072Stable transgenic C9orf72 zebrafish model key aspects of the ALS/FTD phenotype and reveal novel pathological features.Acta Neuropathol Commun2018
28840554RNA Nuclear Export: From Neurological Disorders to Cancer.Adv Exp Med Biol2017
28158451C9ORF72 hexanucleotide repeat exerts toxicity in a stable, inducible motor neuronal cell model, which is rescued by partial depletion of Pten.Hum Mol Genet2017
29076988The Use of Plant-Derived Ribosome Inactivating Proteins in Immunotoxin Development: Past, Present and Future Generations.Toxins (Basel)2017
29170628Targeted Genetic Screen in Amyotrophic Lateral Sclerosis Reveals Novel Genetic Variants with Synergistic Effect on Clinical Phenotype.Front Mol Neurosci2017
29116194Site Specific Modification of Adeno-Associated Virus Enables Both Fluorescent Imaging of Viral Particles and Characterization of the Capsid Interactome.Sci Rep2017
28677678SRSF1-dependent nuclear export inhibition of C9ORF72 repeat transcripts prevents neurodegeneration and associated motor deficits.Nat Commun2017
28714954C9orf72 expansion disrupts ATM-mediated chromosomal break repair.Nat Neurosci2017
27334615The C9orf72 protein interacts with Rab1a and the ULK1 complex to regulate initiation of autophagy.EMBO J2016
25319671Invited review: decoding the pathophysiological mechanisms that underlie RNA dysregulation in neurodegenerative disorders: a review of the current state of the art.Neuropathol Appl Neurobiol2015
26207613Two complementary approaches for intracellular delivery of exogenous enzymes.Sci Rep2015
25943887Antisense RNA foci in the motor neurons of C9ORF72-ALS patients are associated with TDP-43 proteinopathy.Acta Neuropathol2015
26016851C9ORF72 GGGGCC Expanded Repeats Produce Splicing Dysregulation which Correlates with Disease Severity in Amyotrophic Lateral Sclerosis.PLoS One2015
25605962Arginine methylation and citrullination of splicing factor proline- and glutamine-rich (SFPQ/PSF) regulates its association with mRNA.RNA2015
24550725Competitive and cooperative interactions mediate RNA transfer from herpesvirus saimiri ORF57 to the mammalian export adaptor ALYREF.PLoS Pathog2014
25231350In Candida albicans hyphae, Sec2p is physically associated with SEC2 mRNA on secretory vesicles.Mol Microbiol2014
24866055Sequestration of multiple RNA recognition motif-containing proteins by C9orf72 repeat expansions.Brain2014
23299939Chtop is a component of the dynamic TREX mRNA export complex.EMBO J2013
24071927Ribosome-inactivating proteins: potent poisons and molecular tools.Virulence2013
22140111The structure and selectivity of the SR protein SRSF2 RRM domain with RNA.Nucleic Acids Res2012
22706270Drosha regulates neurogenesis by controlling neurogenin 2 expression independent of microRNAs.Nat Neurosci2012
22893130TREX exposes the RNA-binding domain of Nxf1 to enable mRNA export.Nat Commun2012
22817745BLF1, the first Burkholderia pseudomallei toxin, connects inhibition of host protein synthesis with melioidosis.Biochem Soc Trans2012
22480647[Characterisation of Burkholderia pseudomallei Lethal Factor 1 (BLF1). A breakthrough against melioidosis].Med Sci (Paris)2012
20703835The 1H, 13C and 15N backbone and side-chain assignment of the RRM domain of SC35, a regulator of pre-mRNA splicing.Biomol NMR Assign2011
22076380A Burkholderia pseudomallei toxin inhibits helicase activity of translation factor eIF4A.Science2011
21814512An interaction between KSHV ORF57 and UIF provides mRNA-adaptor redundancy in herpesvirus intronless mRNA export.PLoS Pathog2011
21521736FatJ acts via the Hippo mediator Yap1 to restrict the size of neural progenitor cell pools.Development2011
21253573Structural basis for the recognition of cellular mRNA export factor REF by herpes viral proteins HSV-1 ICP27 and HVS ORF57.PLoS Pathog2011
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Collaborators

Sheffield Institute for Translational Neuroscience, University of Sheffield
Co-authored papers 20
Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield
Co-authored papers 17
Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield
Co-authored papers 12
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Co-authored papers 12
Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield
Co-authored papers 12
Institute of Systems, The University of Liverpool
Co-authored papers 9
The University of Manchester
Co-authored papers 8
University of Sheffield
Co-authored papers 2
University of Sheffield
Co-authored papers 2
Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield
Co-authored papers 2
Sheffield Institute for Translational Neuroscience, University of Sheffield
Co-authored papers 1
Center for Genomics and Personalized Medicine, Stanford University School of Medicine
Co-authored papers 1
The University of Edinburgh
Co-authored papers 1
University of California San Francisco
Co-authored papers 1
Emory University
Co-authored papers 1
Sheffield Institute for Translational Neuroscience, University of Sheffield
Co-authored papers 1
Sheffield Institute for Translational Neuroscience, University of Sheffield
Co-authored papers 1
MRC Weatherall Institute of Molecular Medicine, University of Oxford
Co-authored papers 1
Friedrich Miescher Institute for Biomedical Research
Co-authored papers 1
Sheffield Institute for Translational Neuroscience, University of Sheffield
Co-authored papers 1
University of Basel
Co-authored papers 1
University of Sheffield
Co-authored papers 1
Sheffield Institute for Translational Neuroscience, University of Sheffield
Co-authored papers 1
University of Oxford
Co-authored papers 1
Roche Pharmaceutical Research and Early Development, F. Hoffmann-La Roche Ltd
Co-authored papers 1
Stanford University School of Medicine
Co-authored papers 1