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Items: 1 to 50 of 186

1.

Phenotype onset in Huntington's disease knock-in mice is correlated with the incomplete splicing of the mutant huntingtin gene.

Franich NR, Hickey MA, Zhu C, Osborne GF, Ali N, Chu T, Bove NH, Lemesre V, Lerner RP, Zeitlin SO, Howland D, Neueder A, Landles C, Bates GP, Chesselet MF.

J Neurosci Res. 2019 Jul 7. doi: 10.1002/jnr.24493. [Epub ahead of print]

PMID:
31282030
2.

Inhibition of tumour necrosis factor alpha in the R6/2 mouse model of Huntington's disease by etanercept treatment.

Pido-Lopez J, Tanudjojo B, Farag S, Bondulich MK, Andre R, Tabrizi SJ, Bates GP.

Sci Rep. 2019 May 10;9(1):7202. doi: 10.1038/s41598-019-43627-3.

3.

Meso scale discovery-based assays for the detection of aggregated huntingtin.

Reindl W, Baldo B, Schulz J, Janack I, Lindner I, Kleinschmidt M, Sedaghat Y, Thiede C, Tillack K, Schmidt C, Cardaun I, Schwagarus T, Herrmann F, Hotze M, Osborne GF, Herrmann S, Weiss A, Zerbinatti C, Bates GP, Bard J, Munoz-Sanjuan I, Macdonald D.

PLoS One. 2019 Mar 26;14(3):e0213521. doi: 10.1371/journal.pone.0213521. eCollection 2019.

4.

Live-cell super-resolution microscopy reveals a primary role for diffusion in polyglutamine-driven aggresome assembly.

Lu M, Banetta L, Young LJ, Smith EJ, Bates GP, Zaccone A, Kaminski Schierle GS, Tunnacliffe A, Kaminski CF.

J Biol Chem. 2019 Jan 4;294(1):257-268. doi: 10.1074/jbc.RA118.003500. Epub 2018 Nov 6.

5.

FAN1 modifies Huntington's disease progression by stabilizing the expanded HTT CAG repeat.

Goold R, Flower M, Moss DH, Medway C, Wood-Kaczmar A, Andre R, Farshim P, Bates GP, Holmans P, Jones L, Tabrizi SJ.

Hum Mol Genet. 2019 Feb 15;28(4):650-661. doi: 10.1093/hmg/ddy375.

6.

Regulatory mechanisms of incomplete huntingtin mRNA splicing.

Neueder A, Dumas AA, Benjamin AC, Bates GP.

Nat Commun. 2018 Sep 27;9(1):3955. doi: 10.1038/s41467-018-06281-3.

7.

mHTT Seeding Activity: A Marker of Disease Progression and Neurotoxicity in Models of Huntington's Disease.

Ast A, Buntru A, Schindler F, Hasenkopf R, Schulz A, Brusendorf L, Klockmeier K, Grelle G, McMahon B, Niederlechner H, Jansen I, Diez L, Edel J, Boeddrich A, Franklin SA, Baldo B, Schnoegl S, Kunz S, Purfürst B, Gaertner A, Kampinga HH, Morton AJ, Petersén Å, Kirstein J, Bates GP, Wanker EE.

Mol Cell. 2018 Sep 6;71(5):675-688.e6. doi: 10.1016/j.molcel.2018.07.032.

8.

In vivo neutralization of the protagonist role of macrophages during the chronic inflammatory stage of Huntington's disease.

Pido-Lopez J, Andre R, Benjamin AC, Ali N, Farag S, Tabrizi SJ, Bates GP.

Sci Rep. 2018 Jul 30;8(1):11447. doi: 10.1038/s41598-018-29792-x.

9.

Mouse Models of Huntington's Disease.

Farshim PP, Bates GP.

Methods Mol Biol. 2018;1780:97-120. doi: 10.1007/978-1-4939-7825-0_6. Review.

PMID:
29856016
10.

RNA Related Pathology in Huntington's Disease.

Neueder A, Bates GP.

Adv Exp Med Biol. 2018;1049:85-101. doi: 10.1007/978-3-319-71779-1_4. Review.

PMID:
29427099
11.

Stall in Canonical Autophagy-Lysosome Pathways Prompts Nucleophagy-Based Nuclear Breakdown in Neurodegeneration.

Baron O, Boudi A, Dias C, Schilling M, Nölle A, Vizcay-Barrena G, Rattray I, Jungbluth H, Scheper W, Fleck RA, Bates GP, Fanto M.

Curr Biol. 2017 Dec 4;27(23):3626-3642.e6. doi: 10.1016/j.cub.2017.10.054. Epub 2017 Nov 22.

12.

Myostatin inhibition prevents skeletal muscle pathophysiology in Huntington's disease mice.

Bondulich MK, Jolinon N, Osborne GF, Smith EJ, Rattray I, Neueder A, Sathasivam K, Ahmed M, Ali N, Benjamin AC, Chang X, Dick JRT, Ellis M, Franklin SA, Goodwin D, Inuabasi L, Lazell H, Lehar A, Richard-Londt A, Rosinski J, Smith DL, Wood T, Tabrizi SJ, Brandner S, Greensmith L, Howland D, Munoz-Sanjuan I, Lee SJ, Bates GP.

Sci Rep. 2017 Oct 27;7(1):14275. doi: 10.1038/s41598-017-14290-3.

13.

HSF1-dependent and -independent regulation of the mammalian in vivo heat shock response and its impairment in Huntington's disease mouse models.

Neueder A, Gipson TA, Batterton S, Lazell HJ, Farshim PP, Paganetti P, Housman DE, Bates GP.

Sci Rep. 2017 Oct 2;7(1):12556. doi: 10.1038/s41598-017-12897-0.

14.

Disruption to schizophrenia-associated gene Fez1 in the hippocampus of HDAC11 knockout mice.

Bryant DT, Landles C, Papadopoulou AS, Benjamin AC, Duckworth JK, Rosahl T, Benn CL, Bates GP.

Sci Rep. 2017 Sep 19;7(1):11900. doi: 10.1038/s41598-017-11630-1.

15.

The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington's disease patients.

Neueder A, Landles C, Ghosh R, Howland D, Myers RH, Faull RLM, Tabrizi SJ, Bates GP.

Sci Rep. 2017 May 2;7(1):1307. doi: 10.1038/s41598-017-01510-z.

16.

Correlations of Behavioral Deficits with Brain Pathology Assessed through Longitudinal MRI and Histopathology in the HdhQ150/Q150 Mouse Model of Huntington's Disease.

Rattray I, Smith EJ, Crum WR, Walker TA, Gale R, Bates GP, Modo M.

PLoS One. 2017 Jan 18;12(1):e0168556. doi: 10.1371/journal.pone.0168556. eCollection 2017.

17.

Embryonic Mutant Huntingtin Aggregate Formation in Mouse Models of Huntington's Disease.

Osmand AP, Bichell TJ, Bowman AB, Bates GP.

J Huntingtons Dis. 2016 Dec 15;5(4):343-346.

18.

Frequency of nuclear mutant huntingtin inclusion formation in neurons and glia is cell-type-specific.

Jansen AH, van Hal M, Op den Kelder IC, Meier RT, de Ruiter AA, Schut MH, Smith DL, Grit C, Brouwer N, Kamphuis W, Boddeke HW, den Dunnen WF, van Roon WM, Bates GP, Hol EM, Reits EA.

Glia. 2017 Jan;65(1):50-61. doi: 10.1002/glia.23050. Epub 2016 Sep 12.

19.

UBQLN2 Mediates Autophagy-Independent Protein Aggregate Clearance by the Proteasome.

Hjerpe R, Bett JS, Keuss MJ, Solovyova A, McWilliams TG, Johnson C, Sahu I, Varghese J, Wood N, Wightman M, Osborne G, Bates GP, Glickman MH, Trost M, Knebel A, Marchesi F, Kurz T.

Cell. 2016 Aug 11;166(4):935-949. doi: 10.1016/j.cell.2016.07.001. Epub 2016 Jul 28.

20.

The S/T-Rich Motif in the DNAJB6 Chaperone Delays Polyglutamine Aggregation and the Onset of Disease in a Mouse Model.

Kakkar V, Månsson C, de Mattos EP, Bergink S, van der Zwaag M, van Waarde MAWH, Kloosterhuis NJ, Melki R, van Cruchten RTP, Al-Karadaghi S, Arosio P, Dobson CM, Knowles TPJ, Bates GP, van Deursen JM, Linse S, van de Sluis B, Emanuelsson C, Kampinga HH.

Mol Cell. 2016 Apr 21;62(2):272-283. doi: 10.1016/j.molcel.2016.03.017. Epub 2016 Apr 14.

21.

Correction: SIRT1 Activity Is Linked to Its Brain Region-Specific Phosphorylation and Is Impaired in Huntington's Disease Mice.

Tulino R, Benjamin AC, Jolinon N, Smith DL, Chini EN, Carnemolla A, Bates GP.

PLoS One. 2016 Feb 26;11(2):e0150682. doi: 10.1371/journal.pone.0150682. eCollection 2016.

22.

SIRT1 Activity Is Linked to Its Brain Region-Specific Phosphorylation and Is Impaired in Huntington's Disease Mice.

Tulino R, Benjamin AC, Jolinon N, Smith DL, Chini EN, Carnemolla A, Bates GP.

PLoS One. 2016 Jan 27;11(1):e0145425. doi: 10.1371/journal.pone.0145425. eCollection 2016. Erratum in: PLoS One. 2016;11(2):e0150682.

23.

Characterization of Gastric Mucosa Biopsies Reveals Alterations in Huntington's Disease.

McCourt AC, O'Donovan KL, Ekblad E, Sand E, Craufurd D, Rosser A, Sanders D, Stoy N, Rickards H, Wierup N, Bates GP, Björkqvist M, Quarrell O.

PLoS Curr. 2015 Jun 26;7. pii: ecurrents.hd.858b4cc7f235df068387e9c20c436a79. doi: 10.1371/currents.hd.858b4cc7f235df068387e9c20c436a79.

24.

Treating the whole body in Huntington's disease.

Carroll JB, Bates GP, Steffan J, Saft C, Tabrizi SJ.

Lancet Neurol. 2015 Nov;14(11):1135-42. doi: 10.1016/S1474-4422(15)00177-5. Review.

PMID:
26466780
25.

In Vivo Profiling Reveals a Competent Heat Shock Response in Adult Neurons: Implications for Neurodegenerative Disorders.

Carnemolla A, Lazell H, Moussaoui S, Bates GP.

PLoS One. 2015 Jul 2;10(7):e0131985. doi: 10.1371/journal.pone.0131985. eCollection 2015.

26.

Systematic interaction network filtering identifies CRMP1 as a novel suppressor of huntingtin misfolding and neurotoxicity.

Stroedicke M, Bounab Y, Strempel N, Klockmeier K, Yigit S, Friedrich RP, Chaurasia G, Li S, Hesse F, Riechers SP, Russ J, Nicoletti C, Boeddrich A, Wiglenda T, Haenig C, Schnoegl S, Fournier D, Graham RK, Hayden MR, Sigrist S, Bates GP, Priller J, Andrade-Navarro MA, Futschik ME, Wanker EE.

Genome Res. 2015 May;25(5):701-13. doi: 10.1101/gr.182444.114.

27.

HDAC4-myogenin axis as an important marker of HD-related skeletal muscle atrophy.

Mielcarek M, Toczek M, Smeets CJ, Franklin SA, Bondulich MK, Jolinon N, Muller T, Ahmed M, Dick JR, Piotrowska I, Greensmith L, Smolenski RT, Bates GP.

PLoS Genet. 2015 Mar 6;11(3):e1005021. doi: 10.1371/journal.pgen.1005021. eCollection 2015 Mar.

28.

Huntington disease.

Bates GP, Dorsey R, Gusella JF, Hayden MR, Kay C, Leavitt BR, Nance M, Ross CA, Scahill RI, Wetzel R, Wild EJ, Tabrizi SJ.

Nat Rev Dis Primers. 2015 Apr 23;1:15005. doi: 10.1038/nrdp.2015.5. Review.

PMID:
27188817
29.

Characterisation of immune cell function in fragment and full-length Huntington's disease mouse models.

Träger U, Andre R, Magnusson-Lind A, Miller JR, Connolly C, Weiss A, Grueninger S, Silajdžić E, Smith DL, Leavitt BR, Bates GP, Björkqvist M, Tabrizi SJ.

Neurobiol Dis. 2015 Jan;73:388-98. doi: 10.1016/j.nbd.2014.10.012. Epub 2014 Oct 29.

30.

A common gene expression signature in Huntington's disease patient brain regions.

Neueder A, Bates GP.

BMC Med Genomics. 2014 Oct 30;7:60. doi: 10.1186/s12920-014-0060-2.

31.

The Huntington's disease-related cardiomyopathy prevents a hypertrophic response in the R6/2 mouse model.

Mielcarek M, Bondulich MK, Inuabasi L, Franklin SA, Muller T, Bates GP.

PLoS One. 2014 Sep 30;9(9):e108961. doi: 10.1371/journal.pone.0108961. eCollection 2014.

32.

Reducing Igf-1r levels leads to paradoxical and sexually dimorphic effects in HD mice.

Corrochano S, Renna M, Osborne G, Carter S, Stewart M, May J, Bates GP, Brown SD, Rubinsztein DC, Acevedo-Arozena A.

PLoS One. 2014 Aug 20;9(8):e105595. doi: 10.1371/journal.pone.0105595. eCollection 2014.

33.

Dysfunction of the CNS-heart axis in mouse models of Huntington's disease.

Mielcarek M, Inuabasi L, Bondulich MK, Muller T, Osborne GF, Franklin SA, Smith DL, Neueder A, Rosinski J, Rattray I, Protti A, Bates GP.

PLoS Genet. 2014 Aug 7;10(8):e1004550. doi: 10.1371/journal.pgen.1004550. eCollection 2014 Aug.

34.

Genetic deletion of transglutaminase 2 does not rescue the phenotypic deficits observed in R6/2 and zQ175 mouse models of Huntington's disease.

Menalled LB, Kudwa AE, Oakeshott S, Farrar A, Paterson N, Filippov I, Miller S, Kwan M, Olsen M, Beltran J, Torello J, Fitzpatrick J, Mushlin R, Cox K, McConnell K, Mazzella M, He D, Osborne GF, Al-Nackkash R, Bates GP, Tuunanen P, Lehtimaki K, Brunner D, Ghavami A, Ramboz S, Park L, Macdonald D, Munoz-Sanjuan I, Howland D.

PLoS One. 2014 Jun 23;9(6):e99520. doi: 10.1371/journal.pone.0099520. eCollection 2014.

35.

Novel isoforms of heat shock transcription factor 1, HSF1γα and HSF1γβ, regulate chaperone protein gene transcription.

Neueder A, Achilli F, Moussaoui S, Bates GP.

J Biol Chem. 2014 Jul 18;289(29):19894-906. doi: 10.1074/jbc.M114.570739. Epub 2014 May 22.

36.

The PDE1/5 Inhibitor SCH-51866 Does Not Modify Disease Progression in the R6/2 Mouse Model of Huntington's Disease.

Beaumont V, Park L, Rassoulpour A, Dijkman U, Heikkinen T, Lehtimaki K, Kontkanen O, Al Nackkash R, Bates GP, Gleyzes M, Steidl E, Ramboz S, Murphy C, Beconi MG, Dominguez C, Munoz-Sanjuan I.

PLoS Curr. 2014 Feb 13;6. pii: ecurrents.hd.3304e87e460b4bb0dc519a29f4deccca. doi: 10.1371/currents.hd.3304e87e460b4bb0dc519a29f4deccca.

37.

Contesting the dogma of an age-related heat shock response impairment: implications for cardiac-specific age-related disorders.

Carnemolla A, Labbadia JP, Lazell H, Neueder A, Moussaoui S, Bates GP.

Hum Mol Genet. 2014 Jul 15;23(14):3641-56. doi: 10.1093/hmg/ddu073. Epub 2014 Feb 19.

38.

HTT-lowering reverses Huntington's disease immune dysfunction caused by NFκB pathway dysregulation.

Träger U, Andre R, Lahiri N, Magnusson-Lind A, Weiss A, Grueninger S, McKinnon C, Sirinathsinghji E, Kahlon S, Pfister EL, Moser R, Hummerich H, Antoniou M, Bates GP, Luthi-Carter R, Lowdell MW, Björkqvist M, Ostroff GR, Aronin N, Tabrizi SJ.

Brain. 2014 Mar;137(Pt 3):819-33. doi: 10.1093/brain/awt355. Epub 2014 Jan 22.

39.

Correlations of behavioral deficits with brain pathology assessed through longitudinal MRI and histopathology in the R6/1 mouse model of Huntington's disease.

Rattray I, Smith EJ, Crum WR, Walker TA, Gale R, Bates GP, Modo M.

PLoS One. 2013 Dec 19;8(12):e84726. doi: 10.1371/journal.pone.0084726. eCollection 2013.

40.

HDAC4 reduction: a novel therapeutic strategy to target cytoplasmic huntingtin and ameliorate neurodegeneration.

Mielcarek M, Landles C, Weiss A, Bradaia A, Seredenina T, Inuabasi L, Osborne GF, Wadel K, Touller C, Butler R, Robertson J, Franklin SA, Smith DL, Park L, Marks PA, Wanker EE, Olson EN, Luthi-Carter R, van der Putten H, Beaumont V, Bates GP.

PLoS Biol. 2013 Nov;11(11):e1001717. doi: 10.1371/journal.pbio.1001717. Epub 2013 Nov 26.

41.

Dynamic recruitment of active proteasomes into polyglutamine initiated inclusion bodies.

Schipper-Krom S, Juenemann K, Jansen AH, Wiemhoefer A, van den Nieuwendijk R, Smith DL, Hink MA, Bates GP, Overkleeft H, Ovaa H, Reits E.

FEBS Lett. 2014 Jan 3;588(1):151-9. doi: 10.1016/j.febslet.2013.11.023. Epub 2013 Nov 26.

42.

HDAC4 does not act as a protein deacetylase in the postnatal murine brain in vivo.

Mielcarek M, Seredenina T, Stokes MP, Osborne GF, Landles C, Inuabasi L, Franklin SA, Silva JC, Luthi-Carter R, Beaumont V, Bates GP.

PLoS One. 2013 Nov 22;8(11):e80849. doi: 10.1371/journal.pone.0080849. eCollection 2013.

43.

Aberrantly spliced HTT, a new player in Huntington's disease pathogenesis.

Gipson TA, Neueder A, Wexler NS, Bates GP, Housman D.

RNA Biol. 2013 Nov;10(11):1647-52. doi: 10.4161/rna.26706. Epub 2013 Oct 11.

44.

Targeting H3K4 trimethylation in Huntington disease.

Vashishtha M, Ng CW, Yildirim F, Gipson TA, Kratter IH, Bodai L, Song W, Lau A, Labadorf A, Vogel-Ciernia A, Troncosco J, Ross CA, Bates GP, Krainc D, Sadri-Vakili G, Finkbeiner S, Marsh JL, Housman DE, Fraenkel E, Thompson LM.

Proc Natl Acad Sci U S A. 2013 Aug 6;110(32):E3027-36. doi: 10.1073/pnas.1311323110. Epub 2013 Jul 19.

45.

SUMO-2 and PIAS1 modulate insoluble mutant huntingtin protein accumulation.

O'Rourke JG, Gareau JR, Ochaba J, Song W, Raskó T, Reverter D, Lee J, Monteys AM, Pallos J, Mee L, Vashishtha M, Apostol BL, Nicholson TP, Illes K, Zhu YZ, Dasso M, Bates GP, Difiglia M, Davidson B, Wanker EE, Marsh JL, Lima CD, Steffan JS, Thompson LM.

Cell Rep. 2013 Jul 25;4(2):362-75. doi: 10.1016/j.celrep.2013.06.034. Epub 2013 Jul 18.

46.

Correlations of behavioral deficits with brain pathology assessed through longitudinal MRI and histopathology in the R6/2 mouse model of HD.

Rattray I, Smith E, Gale R, Matsumoto K, Bates GP, Modo M.

PLoS One. 2013 Apr 4;8(4):e60012. doi: 10.1371/journal.pone.0060012. Print 2013.

47.

Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease.

Sathasivam K, Neueder A, Gipson TA, Landles C, Benjamin AC, Bondulich MK, Smith DL, Faull RL, Roos RA, Howland D, Detloff PJ, Housman DE, Bates GP.

Proc Natl Acad Sci U S A. 2013 Feb 5;110(6):2366-70. doi: 10.1073/pnas.1221891110. Epub 2013 Jan 22.

48.

Downregulation of cannabinoid receptor 1 from neuropeptide Y interneurons in the basal ganglia of patients with Huntington's disease and mouse models.

Horne EA, Coy J, Swinney K, Fung S, Cherry AE, Marrs WR, Naydenov AV, Lin YH, Sun X, Keene CD, Grouzmann E, Muchowski P, Bates GP, Mackie K, Stella N.

Eur J Neurosci. 2013 Feb;37(3):429-40. doi: 10.1111/ejn.12045. Epub 2012 Nov 21.

49.

Mutant huntingtin fragmentation in immune cells tracks Huntington's disease progression.

Weiss A, Träger U, Wild EJ, Grueninger S, Farmer R, Landles C, Scahill RI, Lahiri N, Haider S, Macdonald D, Frost C, Bates GP, Bilbe G, Kuhn R, Andre R, Tabrizi SJ.

J Clin Invest. 2012 Oct;122(10):3731-6. doi: 10.1172/JCI64565. Epub 2012 Sep 17.

50.

Fragments of HdhQ150 mutant huntingtin form a soluble oligomer pool that declines with aggregate deposition upon aging.

Marcellin D, Abramowski D, Young D, Richter J, Weiss A, Marcel A, Maassen J, Kauffmann M, Bibel M, Shimshek DR, Faull RL, Bates GP, Kuhn RR, Van der Putten PH, Schmid P, Lotz GP.

PLoS One. 2012;7(9):e44457. doi: 10.1371/journal.pone.0044457. Epub 2012 Sep 12.

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