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Mutations in the Glycosyltransferase Domain of GLT8D1 Are Associated with Familial Amyotrophic Lateral Sclerosis.

Cooper-Knock J, Moll T, Ramesh T, Castelli L, Beer A, Robins H, Fox I, Niedermoser I, Van Damme P, Moisse M, Robberecht W, Hardiman O, Panades MP, Assialioui A, Mora JS, Basak AN, Morrison KE, Shaw CE, Al-Chalabi A, Landers JE, Wyles M, Heath PR, Higginbottom A, Walsh T, Kazoka M, McDermott CJ, Hautbergue GM, Kirby J, Shaw PJ.

Cell Rep. 2019 Feb 26;26(9):2298-2306.e5. doi: 10.1016/j.celrep.2019.02.006.


Micro-RNAs secreted through astrocyte-derived extracellular vesicles cause neuronal network degeneration in C9orf72 ALS.

Varcianna A, Myszczynska MA, Castelli LM, O'Neill B, Kim Y, Talbot J, Nyberg S, Nyamali I, Heath PR, Stopford MJ, Hautbergue GM, Ferraiuolo L.

EBioMedicine. 2019 Feb;40:626-635. doi: 10.1016/j.ebiom.2018.11.067. Epub 2019 Jan 31.


Astrocyte adenosine deaminase loss increases motor neuron toxicity in amyotrophic lateral sclerosis.

Allen SP, Hall B, Castelli LM, Francis L, Woof R, Siskos AP, Kouloura E, Gray E, Thompson AG, Talbot K, Higginbottom A, Myszczynska M, Allen CF, Stopford MJ, Hemingway J, Bauer CS, Webster CP, De Vos KJ, Turner MR, Keun HC, Hautbergue GM, Ferraiuolo L, Shaw PJ.

Brain. 2019 Mar 1;142(3):586-605. doi: 10.1093/brain/awy353.


Stable transgenic C9orf72 zebrafish model key aspects of the ALS/FTD phenotype and reveal novel pathological features.

Shaw MP, Higginbottom A, McGown A, Castelli LM, James E, Hautbergue GM, Shaw PJ, Ramesh TM.

Acta Neuropathol Commun. 2018 Nov 19;6(1):125. doi: 10.1186/s40478-018-0629-7.


TIGAR inclusion pathology is specific for Lewy body diseases.

López KLR, Simpson JE, Watson LC, Mortiboys H, Hautbergue GM, Bandmann O, Highley JR.

Brain Res. 2019 Mar 1;1706:218-223. doi: 10.1016/j.brainres.2018.09.032. Epub 2018 Sep 26.


Translating SOD1 Gene Silencing toward the Clinic: A Highly Efficacious, Off-Target-free, and Biomarker-Supported Strategy for fALS.

Iannitti T, Scarrott JM, Likhite S, Coldicott IRP, Lewis KE, Heath PR, Higginbottom A, Myszczynska MA, Milo M, Hautbergue GM, Meyer K, Kaspar BK, Ferraiuolo L, Shaw PJ, Azzouz M.

Mol Ther Nucleic Acids. 2018 Sep 7;12:75-88. doi: 10.1016/j.omtn.2018.04.015. Epub 2018 May 3.


Burkholderia Lethal Factor 1, a Novel Anti-Cancer Toxin, Demonstrates Selective Cytotoxicity in MYCN-Amplified Neuroblastoma Cells.

Rust A, Shah S, Hautbergue GM, Davletov B.

Toxins (Basel). 2018 Jun 27;10(7). pii: E261. doi: 10.3390/toxins10070261.


Proteomic and cellular localisation studies suggest non-tight junction cytoplasmic and nuclear roles for occludin in astrocytes.

Morgan SV, Garwood CJ, Jennings L, Simpson JE, Castelli LM, Heath PR, Mihaylov SR, Vaquéz-Villaseñor I, Minshull TC, Ince PG, Dickman MJ, Hautbergue GM, Wharton SB.

Eur J Neurosci. 2018 Jun;47(12):1444-1456. doi: 10.1111/ejn.13933. Epub 2018 May 30.


Targeted Genetic Screen in Amyotrophic Lateral Sclerosis Reveals Novel Genetic Variants with Synergistic Effect on Clinical Phenotype.

Cooper-Knock J, Robins H, Niedermoser I, Wyles M, Heath PR, Higginbottom A, Walsh T, Kazoka M; Project MinE ALS Sequencing Consortium, Ince PG, Hautbergue GM, McDermott CJ, Kirby J, Shaw PJ.

Front Mol Neurosci. 2017 Nov 9;10:370. doi: 10.3389/fnmol.2017.00370. eCollection 2017.


Site Specific Modification of Adeno-Associated Virus Enables Both Fluorescent Imaging of Viral Particles and Characterization of the Capsid Interactome.

Chandran JS, Sharp PS, Karyka E, Aves-Cruzeiro JMDC, Coldicott I, Castelli L, Hautbergue G, Collins MO, Azzouz M.

Sci Rep. 2017 Nov 7;7(1):14766. doi: 10.1038/s41598-017-15255-2.


The Use of Plant-Derived Ribosome Inactivating Proteins in Immunotoxin Development: Past, Present and Future Generations.

Rust A, Partridge LJ, Davletov B, Hautbergue GM.

Toxins (Basel). 2017 Oct 27;9(11). pii: E344. doi: 10.3390/toxins9110344. Review.


RNA Nuclear Export: From Neurological Disorders to Cancer.

Hautbergue GM.

Adv Exp Med Biol. 2017;1007:89-109. doi: 10.1007/978-3-319-60733-7_6. Review.


C9orf72 expansion disrupts ATM-mediated chromosomal break repair.

Walker C, Herranz-Martin S, Karyka E, Liao C, Lewis K, Elsayed W, Lukashchuk V, Chiang SC, Ray S, Mulcahy PJ, Jurga M, Tsagakis I, Iannitti T, Chandran J, Coldicott I, De Vos KJ, Hassan MK, Higginbottom A, Shaw PJ, Hautbergue GM, Azzouz M, El-Khamisy SF.

Nat Neurosci. 2017 Sep;20(9):1225-1235. doi: 10.1038/nn.4604. Epub 2017 Jul 17.


SRSF1-dependent nuclear export inhibition of C9ORF72 repeat transcripts prevents neurodegeneration and associated motor deficits.

Hautbergue GM, Castelli LM, Ferraiuolo L, Sanchez-Martinez A, Cooper-Knock J, Higginbottom A, Lin YH, Bauer CS, Dodd JE, Myszczynska MA, Alam SM, Garneret P, Chandran JS, Karyka E, Stopford MJ, Smith EF, Kirby J, Meyer K, Kaspar BK, Isaacs AM, El-Khamisy SF, De Vos KJ, Ning K, Azzouz M, Whitworth AJ, Shaw PJ.

Nat Commun. 2017 Jul 5;8:16063. doi: 10.1038/ncomms16063.


C9ORF72 hexanucleotide repeat exerts toxicity in a stable, inducible motor neuronal cell model, which is rescued by partial depletion of Pten.

Stopford MJ, Higginbottom A, Hautbergue GM, Cooper-Knock J, Mulcahy PJ, De Vos KJ, Renton AE, Pliner H, Calvo A, Chio A, Traynor BJ, Azzouz M, Heath PR; ITALSGEN Consortium, NeuroX Consortium, Kirby J, Shaw PJ.

Hum Mol Genet. 2017 Mar 15;26(6):1133-1145. doi: 10.1093/hmg/ddx022.


The C9orf72 protein interacts with Rab1a and the ULK1 complex to regulate initiation of autophagy.

Webster CP, Smith EF, Bauer CS, Moller A, Hautbergue GM, Ferraiuolo L, Myszczynska MA, Higginbottom A, Walsh MJ, Whitworth AJ, Kaspar BK, Meyer K, Shaw PJ, Grierson AJ, De Vos KJ.

EMBO J. 2016 Aug 1;35(15):1656-76. doi: 10.15252/embj.201694401. Epub 2016 Jun 22.


Two complementary approaches for intracellular delivery of exogenous enzymes.

Rust A, Hassan HH, Sedelnikova S, Niranjan D, Hautbergue G, Abbas SA, Partridge L, Rice D, Binz T, Davletov B.

Sci Rep. 2015 Jul 24;5:12444. doi: 10.1038/srep12444.


C9ORF72 GGGGCC Expanded Repeats Produce Splicing Dysregulation which Correlates with Disease Severity in Amyotrophic Lateral Sclerosis.

Cooper-Knock J, Bury JJ, Heath PR, Wyles M, Higginbottom A, Gelsthorpe C, Highley JR, Hautbergue G, Rattray M, Kirby J, Shaw PJ.

PLoS One. 2015 May 27;10(5):e0127376. doi: 10.1371/journal.pone.0127376. eCollection 2015.


Antisense RNA foci in the motor neurons of C9ORF72-ALS patients are associated with TDP-43 proteinopathy.

Cooper-Knock J, Higginbottom A, Stopford MJ, Highley JR, Ince PG, Wharton SB, Pickering-Brown S, Kirby J, Hautbergue GM, Shaw PJ.

Acta Neuropathol. 2015 Jul;130(1):63-75. doi: 10.1007/s00401-015-1429-9. Epub 2015 May 6.


Arginine methylation and citrullination of splicing factor proline- and glutamine-rich (SFPQ/PSF) regulates its association with mRNA.

Snijders AP, Hautbergue GM, Bloom A, Williamson JC, Minshull TC, Phillips HL, Mihaylov SR, Gjerde DT, Hornby DP, Wilson SA, Hurd PJ, Dickman MJ.

RNA. 2015 Mar;21(3):347-59. doi: 10.1261/rna.045138.114. Epub 2015 Jan 20.


Invited review: decoding the pathophysiological mechanisms that underlie RNA dysregulation in neurodegenerative disorders: a review of the current state of the art.

Walsh MJ, Cooper-Knock J, Dodd JE, Stopford MJ, Mihaylov SR, Kirby J, Shaw PJ, Hautbergue GM.

Neuropathol Appl Neurobiol. 2015 Feb;41(2):109-34. doi: 10.1111/nan.12187. Review.


In Candida albicans hyphae, Sec2p is physically associated with SEC2 mRNA on secretory vesicles.

Caballero-Lima D, Hautbergue GM, Wilson SA, Sudbery PE.

Mol Microbiol. 2014 Nov;94(4):828-42. doi: 10.1111/mmi.12799. Epub 2014 Oct 14.


Sequestration of multiple RNA recognition motif-containing proteins by C9orf72 repeat expansions.

Cooper-Knock J, Walsh MJ, Higginbottom A, Robin Highley J, Dickman MJ, Edbauer D, Ince PG, Wharton SB, Wilson SA, Kirby J, Hautbergue GM, Shaw PJ.

Brain. 2014 Jul;137(Pt 7):2040-51. doi: 10.1093/brain/awu120. Epub 2014 May 27.


Competitive and cooperative interactions mediate RNA transfer from herpesvirus saimiri ORF57 to the mammalian export adaptor ALYREF.

Tunnicliffe RB, Hautbergue GM, Wilson SA, Kalra P, Golovanov AP.

PLoS Pathog. 2014 Feb 13;10(2):e1003907. doi: 10.1371/journal.ppat.1003907. eCollection 2014 Feb.


Ribosome-inactivating proteins: potent poisons and molecular tools.

Walsh MJ, Dodd JE, Hautbergue GM.

Virulence. 2013 Nov 15;4(8):774-84. doi: 10.4161/viru.26399. Epub 2013 Sep 17. Review.


Chtop is a component of the dynamic TREX mRNA export complex.

Chang CT, Hautbergue GM, Walsh MJ, Viphakone N, van Dijk TB, Philipsen S, Wilson SA.

EMBO J. 2013 Feb 6;32(3):473-86. doi: 10.1038/emboj.2012.342. Epub 2013 Jan 8.


TREX exposes the RNA-binding domain of Nxf1 to enable mRNA export.

Viphakone N, Hautbergue GM, Walsh M, Chang CT, Holland A, Folco EG, Reed R, Wilson SA.

Nat Commun. 2012;3:1006. doi: 10.1038/ncomms2005.


BLF1, the first Burkholderia pseudomallei toxin, connects inhibition of host protein synthesis with melioidosis.

Hautbergue GM, Wilson SA.

Biochem Soc Trans. 2012 Aug;40(4):842-5. doi: 10.1042/BST20120057. Review.


Drosha regulates neurogenesis by controlling neurogenin 2 expression independent of microRNAs.

Knuckles P, Vogt MA, Lugert S, Milo M, Chong MM, Hautbergue GM, Wilson SA, Littman DR, Taylor V.

Nat Neurosci. 2012 Jun 17;15(7):962-9. doi: 10.1038/nn.3139.


[Characterisation of Burkholderia pseudomallei Lethal Factor 1 (BLF1). A breakthrough against melioidosis].

Hautbergue G.

Med Sci (Paris). 2012 Mar;28(3):262-4. doi: 10.1051/medsci/2012283011. Epub 2012 Apr 6. French. No abstract available.


The structure and selectivity of the SR protein SRSF2 RRM domain with RNA.

Phelan MM, Goult BT, Clayton JC, Hautbergue GM, Wilson SA, Lian LY.

Nucleic Acids Res. 2012 Apr;40(7):3232-44. doi: 10.1093/nar/gkr1164. Epub 2011 Dec 2.


A Burkholderia pseudomallei toxin inhibits helicase activity of translation factor eIF4A.

Cruz-Migoni A, Hautbergue GM, Artymiuk PJ, Baker PJ, Bokori-Brown M, Chang CT, Dickman MJ, Essex-Lopresti A, Harding SV, Mahadi NM, Marshall LE, Mobbs GW, Mohamed R, Nathan S, Ngugi SA, Ong C, Ooi WF, Partridge LJ, Phillips HL, Raih MF, Ruzheinikov S, Sarkar-Tyson M, Sedelnikova SE, Smither SJ, Tan P, Titball RW, Wilson SA, Rice DW.

Science. 2011 Nov 11;334(6057):821-4. doi: 10.1126/science.1211915.


An interaction between KSHV ORF57 and UIF provides mRNA-adaptor redundancy in herpesvirus intronless mRNA export.

Jackson BR, Boyne JR, Noerenberg M, Taylor A, Hautbergue GM, Walsh MJ, Wheat R, Blackbourn DJ, Wilson SA, Whitehouse A.

PLoS Pathog. 2011 Jul;7(7):e1002138. doi: 10.1371/journal.ppat.1002138. Epub 2011 Jul 21.


FatJ acts via the Hippo mediator Yap1 to restrict the size of neural progenitor cell pools.

Van Hateren NJ, Das RM, Hautbergue GM, Borycki AG, Placzek M, Wilson SA.

Development. 2011 May;138(10):1893-902. doi: 10.1242/dev.064204.


Structural basis for the recognition of cellular mRNA export factor REF by herpes viral proteins HSV-1 ICP27 and HVS ORF57.

Tunnicliffe RB, Hautbergue GM, Kalra P, Jackson BR, Whitehouse A, Wilson SA, Golovanov AP.

PLoS Pathog. 2011 Jan 6;7(1):e1001244. doi: 10.1371/journal.ppat.1001244.


The 1H, 13C and 15N backbone and side-chain assignment of the RRM domain of SC35, a regulator of pre-mRNA splicing.

Clayton JC, Phelan M, Goult BT, Hautbergue GM, Wilson SA, Lian LY.

Biomol NMR Assign. 2011 Apr;5(1):7-10. doi: 10.1007/s12104-010-9254-5. Epub 2010 Aug 12.


Arginine methylation of REF/ALY promotes efficient handover of mRNA to TAP/NXF1.

Hung ML, Hautbergue GM, Snijders AP, Dickman MJ, Wilson SA.

Nucleic Acids Res. 2010 Jun;38(10):3351-61. doi: 10.1093/nar/gkq033. Epub 2010 Feb 2.


Structure and function of mRNA export adaptors.

Walsh MJ, Hautbergue GM, Wilson SA.

Biochem Soc Trans. 2010 Feb;38(Pt 1):232-6. doi: 10.1042/BST0380232.


UIF, a New mRNA export adaptor that works together with REF/ALY, requires FACT for recruitment to mRNA.

Hautbergue GM, Hung ML, Walsh MJ, Snijders AP, Chang CT, Jones R, Ponting CP, Dickman MJ, Wilson SA.

Curr Biol. 2009 Dec 1;19(22):1918-24. doi: 10.1016/j.cub.2009.09.041.


Mutually exclusive interactions drive handover of mRNA from export adaptors to TAP.

Hautbergue GM, Hung ML, Golovanov AP, Lian LY, Wilson SA.

Proc Natl Acad Sci U S A. 2008 Apr 1;105(13):5154-9. doi: 10.1073/pnas.0709167105. Epub 2008 Mar 25.


Increasing the sensitivity of cryoprobe protein NMR experiments by using the sole low-conductivity arginine glutamate salt.

Hautbergue GM, Golovanov AP.

J Magn Reson. 2008 Apr;191(2):335-9. doi: 10.1016/j.jmr.2007.12.017. Epub 2008 Jan 3.


Structural and functional analysis of RNA and TAP binding to SF2/ASF.

Tintaru AM, Hautbergue GM, Hounslow AM, Hung ML, Lian LY, Craven CJ, Wilson SA.

EMBO Rep. 2007 Aug;8(8):756-62. Epub 2007 Jul 13.


Assignment of 1H, 13C, and 15N resonances for SF2 RNA recognition motif 2.

Tintaru AM, Hautbergue GM, Hounslow AM, Lian LY, Wilson SA, Craven CJ.

J Biomol NMR. 2007 Jun;38(2):193. Epub 2007 Apr 6. No abstract available.


Molecular basis of RNA recognition and TAP binding by the SR proteins SRp20 and 9G8.

Hargous Y, Hautbergue GM, Tintaru AM, Skrisovska L, Golovanov AP, Stevenin J, Lian LY, Wilson SA, Allain FH.

EMBO J. 2006 Nov 1;25(21):5126-37. Epub 2006 Oct 12.


The solution structure of REF2-I reveals interdomain interactions and regions involved in binding mRNA export factors and RNA.

Golovanov AP, Hautbergue GM, Tintaru AM, Lian LY, Wilson SA.

RNA. 2006 Nov;12(11):1933-48. Epub 2006 Sep 25.


Assignment of 1H, 13C, and 15N resonances for the REF2-I mRNA export factor.

Golovanov AP, Hautbergue GM, Wilson SA, Lian LY.

J Biomol NMR. 2006;36 Suppl 1:41. No abstract available.


The prototype gamma-2 herpesvirus nucleocytoplasmic shuttling protein, ORF 57, transports viral RNA through the cellular mRNA export pathway.

Williams BJ, Boyne JR, Goodwin DJ, Roaden L, Hautbergue GM, Wilson SA, Whitehouse A.

Biochem J. 2005 Apr 15;387(Pt 2):295-308.


CTD kinase I is involved in RNA polymerase I transcription.

Bouchoux C, Hautbergue G, Grenetier S, Carles C, Riva M, Goguel V.

Nucleic Acids Res. 2004 Nov 1;32(19):5851-60. Print 2004.


A simple method for improving protein solubility and long-term stability.

Golovanov AP, Hautbergue GM, Wilson SA, Lian LY.

J Am Chem Soc. 2004 Jul 28;126(29):8933-9.


Activation of the cyclin-dependent kinase CTDK-I requires the heterodimerization of two unstable subunits.

Hautbergue G, Goguel V.

J Biol Chem. 2001 Mar 16;276(11):8005-13. Epub 2000 Dec 15.

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