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Items: 1 to 50 of 96

1.

Electrocardiogram Abnormalities Suggest Aberrant Cardiac Conduction in Huntington's Disease.

Stephen CD, Hung J, Schifitto G, Hersch SM, Rosas HD.

Mov Disord Clin Pract. 2018 Apr 11;5(3):306-311. doi: 10.1002/mdc3.12596. eCollection 2018 May-Jun.

2.

Complex spatial and temporally defined myelin and axonal degeneration in Huntington disease.

Rosas HD, Wilkens P, Salat DH, Mercaldo ND, Vangel M, Yendiki AY, Hersch SM.

Neuroimage Clin. 2018 Feb 19;20:236-242. doi: 10.1016/j.nicl.2018.01.029. eCollection 2018.

3.

The CREST-E study of creatine for Huntington disease: A randomized controlled trial.

Hersch SM, Schifitto G, Oakes D, Bredlau AL, Meyers CM, Nahin R, Rosas HD; Huntington Study Group CREST-E Investigators and Coordinators.

Neurology. 2017 Aug 8;89(6):594-601. doi: 10.1212/WNL.0000000000004209. Epub 2017 Jul 12.

4.

KEAP1-modifying small molecule reveals muted NRF2 signaling responses in neural stem cells from Huntington's disease patients.

Quinti L, Dayalan Naidu S, Träger U, Chen X, Kegel-Gleason K, Llères D, Connolly C, Chopra V, Low C, Moniot S, Sapp E, Tousley AR, Vodicka P, Van Kanegan MJ, Kaltenbach LS, Crawford LA, Fuszard M, Higgins M, Miller JRC, Farmer RE, Potluri V, Samajdar S, Meisel L, Zhang N, Snyder A, Stein R, Hersch SM, Ellerby LM, Weerapana E, Schwarzschild MA, Steegborn C, Leavitt BR, Degterev A, Tabrizi SJ, Lo DC, DiFiglia M, Thompson LM, Dinkova-Kostova AT, Kazantsev AG.

Proc Natl Acad Sci U S A. 2017 Jun 6;114(23):E4676-E4685. doi: 10.1073/pnas.1614943114. Epub 2017 May 22.

5.

A systems-level "misunderstanding": the plasma metabolome in Huntington's disease.

Rosas HD, Doros G, Bhasin S, Thomas B, Gevorkian S, Malarick K, Matson W, Hersch SM.

Ann Clin Transl Neurol. 2015 Jul;2(7):756-68. doi: 10.1002/acn3.214. Epub 2015 May 28.

6.

PRECREST: a phase II prevention and biomarker trial of creatine in at-risk Huntington disease.

Rosas HD, Doros G, Gevorkian S, Malarick K, Reuter M, Coutu JP, Triggs TD, Wilkens PJ, Matson W, Salat DH, Hersch SM.

Neurology. 2014 Mar 11;82(10):850-7. doi: 10.1212/WNL.0000000000000187. Epub 2014 Feb 7.

7.

HTRF analysis of soluble huntingtin in PHAROS PBMCs.

Moscovitch-Lopatin M, Goodman RE, Eberly S, Ritch JJ, Rosas HD, Matson S, Matson W, Oakes D, Young AB, Shoulson I, Hersch SM; Huntington Study Group PHAROS Investigators.

Neurology. 2013 Sep 24;81(13):1134-40. doi: 10.1212/WNL.0b013e3182a55ede. Epub 2013 Aug 21.

8.

Candidate glutamatergic and dopaminergic pathway gene variants do not influence Huntington's disease motor onset.

Ramos EM, Latourelle JC, Gillis T, Mysore JS, Squitieri F, Di Pardo A, Di Donato S, Gellera C, Hayden MR, Morrison PJ, Nance M, Ross CA, Margolis RL, Gomez-Tortosa E, Ayuso C, Suchowersky O, Trent RJ, McCusker E, Novelletto A, Frontali M, Jones R, Ashizawa T, Frank S, Saint-Hilaire MH, Hersch SM, Rosas HD, Lucente D, Harrison MB, Zanko A, Abramson RK, Marder K, Gusella JF, Lee JM, Alonso I, Sequeiros J, Myers RH, Macdonald ME.

Neurogenetics. 2013 Nov;14(3-4):173-9. doi: 10.1007/s10048-013-0364-y. Epub 2013 May 4.

9.

Population stratification may bias analysis of PGC-1α as a modifier of age at Huntington disease motor onset.

Ramos EM, Latourelle JC, Lee JH, Gillis T, Mysore JS, Squitieri F, Di Pardo A, Di Donato S, Hayden MR, Morrison PJ, Nance M, Ross CA, Margolis RL, Gomez-Tortosa E, Ayuso C, Suchowersky O, Trent RJ, McCusker E, Novelletto A, Frontali M, Jones R, Ashizawa T, Frank S, Saint-Hilaire MH, Hersch SM, Rosas HD, Lucente D, Harrison MB, Zanko A, Marder K, Gusella JF, Lee JM, Alonso I, Sequeiros J, Myers RH, Macdonald ME.

Hum Genet. 2012 Dec;131(12):1833-40. doi: 10.1007/s00439-012-1205-z. Epub 2012 Jul 25.

10.

TAA repeat variation in the GRIK2 gene does not influence age at onset in Huntington's disease.

Lee JH, Lee JM, Ramos EM, Gillis T, Mysore JS, Kishikawa S, Hadzi T, Hendricks AE, Hayden MR, Morrison PJ, Nance M, Ross CA, Margolis RL, Squitieri F, Gellera C, Gomez-Tortosa E, Ayuso C, Suchowersky O, Trent RJ, McCusker E, Novelletto A, Frontali M, Jones R, Ashizawa T, Frank S, Saint-Hilaire MH, Hersch SM, Rosas HD, Lucente D, Harrison MB, Zanko A, Abramson RK, Marder K, Sequeiros J, Landwehrmeyer GB; Registry Study of the European Huntington's Disease Network, Shoulson I; Huntington Study Group COHORT project, Myers RH, MacDonald ME, Gusella JF.

Biochem Biophys Res Commun. 2012 Aug 3;424(3):404-8. Epub 2012 Jul 3.

11.

Alterations in brain transition metals in Huntington disease: an evolving and intricate story.

Rosas HD, Chen YI, Doros G, Salat DH, Chen NK, Kwong KK, Bush A, Fox J, Hersch SM.

Arch Neurol. 2012 Jul;69(7):887-93.

12.

Common SNP-based haplotype analysis of the 4p16.3 Huntington disease gene region.

Lee JM, Gillis T, Mysore JS, Ramos EM, Myers RH, Hayden MR, Morrison PJ, Nance M, Ross CA, Margolis RL, Squitieri F, Griguoli A, Di Donato S, Gomez-Tortosa E, Ayuso C, Suchowersky O, Trent RJ, McCusker E, Novelletto A, Frontali M, Jones R, Ashizawa T, Frank S, Saint-Hilaire MH, Hersch SM, Rosas HD, Lucente D, Harrison MB, Zanko A, Abramson RK, Marder K, Sequeiros J, MacDonald ME, Gusella JF.

Am J Hum Genet. 2012 Mar 9;90(3):434-44. doi: 10.1016/j.ajhg.2012.01.005. Epub 2012 Mar 1.

13.

CAG repeat expansion in Huntington disease determines age at onset in a fully dominant fashion.

Lee JM, Ramos EM, Lee JH, Gillis T, Mysore JS, Hayden MR, Warby SC, Morrison P, Nance M, Ross CA, Margolis RL, Squitieri F, Orobello S, Di Donato S, Gomez-Tortosa E, Ayuso C, Suchowersky O, Trent RJ, McCusker E, Novelletto A, Frontali M, Jones R, Ashizawa T, Frank S, Saint-Hilaire MH, Hersch SM, Rosas HD, Lucente D, Harrison MB, Zanko A, Abramson RK, Marder K, Sequeiros J, Paulsen JS; PREDICT-HD study of the Huntington Study Group (HSG), Landwehrmeyer GB; REGISTRY study of the European Huntington's Disease Network, Myers RH; HD-MAPS Study Group, MacDonald ME, Gusella JF; COHORT study of the HSG.

Neurology. 2012 Mar 6;78(10):690-5. doi: 10.1212/WNL.0b013e318249f683. Epub 2012 Feb 8.

14.

Transcriptional modulator H2A histone family, member Y (H2AFY) marks Huntington disease activity in man and mouse.

Hu Y, Chopra V, Chopra R, Locascio JJ, Liao Z, Ding H, Zheng B, Matson WR, Ferrante RJ, Rosas HD, Hersch SM, Scherzer CR.

Proc Natl Acad Sci U S A. 2011 Oct 11;108(41):17141-6. doi: 10.1073/pnas.1104409108. Epub 2011 Oct 3.

15.

Biomarkers to Enable the Development of Neuroprotective Therapies for Huntington’s Disease.

Hersch SM, Rosas HD.

In: Lo DC, Hughes RE, editors. Neurobiology of Huntington's Disease: Applications to Drug Discovery. Boca Raton (FL): CRC Press/Taylor & Francis; 2011. Chapter 11.

16.

The Sirtuin 2 microtubule deacetylase is an abundant neuronal protein that accumulates in the aging CNS.

Maxwell MM, Tomkinson EM, Nobles J, Wizeman JW, Amore AM, Quinti L, Chopra V, Hersch SM, Kazantsev AG.

Hum Mol Genet. 2011 Oct 15;20(20):3986-96. doi: 10.1093/hmg/ddr326. Epub 2011 Jul 26.

17.

A tale of two factors: what determines the rate of progression in Huntington's disease? A longitudinal MRI study.

Rosas HD, Reuter M, Doros G, Lee SY, Triggs T, Malarick K, Fischl B, Salat DH, Hersch SM.

Mov Disord. 2011 Aug 1;26(9):1691-7. doi: 10.1002/mds.23762. Epub 2011 May 24.

18.

Cysteine oxidation within N-terminal mutant huntingtin promotes oligomerization and delays clearance of soluble protein.

Fox JH, Connor T, Stiles M, Kama J, Lu Z, Dorsey K, Lieberman G, Sapp E, Cherny RA, Banks M, Volitakis I, DiFiglia M, Berezovska O, Bush AI, Hersch SM.

J Biol Chem. 2011 May 20;286(20):18320-30. doi: 10.1074/jbc.M110.199448. Epub 2011 Mar 30. Erratum in: J Biol Chem. 2011 Jul 29;286(30):27068. Liebermann, Gregory [corrected to Lieberman, Gregory].

19.

The mTOR kinase inhibitor Everolimus decreases S6 kinase phosphorylation but fails to reduce mutant huntingtin levels in brain and is not neuroprotective in the R6/2 mouse model of Huntington's disease.

Fox JH, Connor T, Chopra V, Dorsey K, Kama JA, Bleckmann D, Betschart C, Hoyer D, Frentzel S, Difiglia M, Paganetti P, Hersch SM.

Mol Neurodegener. 2010 Jun 22;5:26. doi: 10.1186/1750-1326-5-26.

20.

Reduced creatine kinase as a central and peripheral biomarker in Huntington's disease.

Kim J, Amante DJ, Moody JP, Edgerly CK, Bordiuk OL, Smith K, Matson SA, Matson WR, Scherzer CR, Rosas HD, Hersch SM, Ferrante RJ.

Biochim Biophys Acta. 2010 Jul-Aug;1802(7-8):673-81. doi: 10.1016/j.bbadis.2010.05.001. Epub 2010 May 9.

21.

Altered white matter microstructure in the corpus callosum in Huntington's disease: implications for cortical "disconnection".

Rosas HD, Lee SY, Bender AC, Zaleta AK, Vangel M, Yu P, Fischl B, Pappu V, Onorato C, Cha JH, Salat DH, Hersch SM.

Neuroimage. 2010 Feb 15;49(4):2995-3004. doi: 10.1016/j.neuroimage.2009.10.015. Epub 2009 Oct 19.

22.

Complexity and heterogeneity: what drives the ever-changing brain in Huntington's disease?

Rosas HD, Salat DH, Lee SY, Zaleta AK, Hevelone N, Hersch SM.

Ann N Y Acad Sci. 2008 Dec;1147:196-205. doi: 10.1196/annals.1427.034. Review.

23.

Repeat instability in the 27-39 CAG range of the HD gene in the Venezuelan kindreds: Counseling implications.

Brocklebank D, Gayán J, Andresen JM, Roberts SA, Young AB, Snodgrass SR, Penney JB, Ramos-Arroyo MA, Cha JJ, Rosas HD, Hersch SM, Feigin A, Cherny SS, Wexler NS, Housman DE, Cardon LR; International-Venezuela Collaborative Research Group.

Am J Med Genet B Neuropsychiatr Genet. 2009 Apr 5;150B(3):425-9. doi: 10.1002/ajmg.b.30826.

24.

Neuroprotection for Huntington's disease: ready, set, slow.

Hersch SM, Rosas HD.

Neurotherapeutics. 2008 Apr;5(2):226-36. doi: 10.1016/j.nurt.2008.01.003. Review.

25.

Cerebral cortex and the clinical expression of Huntington's disease: complexity and heterogeneity.

Rosas HD, Salat DH, Lee SY, Zaleta AK, Pappu V, Fischl B, Greve D, Hevelone N, Hersch SM.

Brain. 2008 Apr;131(Pt 4):1057-68. doi: 10.1093/brain/awn025. Epub 2008 Mar 12.

26.

Neuroprotective effects of synaptic modulation in Huntington's disease R6/2 mice.

Stack EC, Dedeoglu A, Smith KM, Cormier K, Kubilus JK, Bogdanov M, Matson WR, Yang L, Jenkins BG, Luthi-Carter R, Kowall NW, Hersch SM, Beal MF, Ferrante RJ.

J Neurosci. 2007 Nov 21;27(47):12908-15.

27.

Histones associated with downregulated genes are hypo-acetylated in Huntington's disease models.

Sadri-Vakili G, Bouzou B, Benn CL, Kim MO, Chawla P, Overland RP, Glajch KE, Xia E, Qiu Z, Hersch SM, Clark TW, Yohrling GJ, Cha JH.

Hum Mol Genet. 2007 Jun 1;16(11):1293-306. Epub 2007 Apr 4.

PMID:
17409194
28.

Drug targeting of dysregulated transcription in Huntington's disease.

Kazantsev AG, Hersch SM.

Prog Neurobiol. 2007 Nov;83(4):249-59. Epub 2007 Feb 23. Review.

29.

Genome-wide significance for a modifier of age at neurological onset in Huntington's disease at 6q23-24: the HD MAPS study.

Li JL, Hayden MR, Warby SC, Durr A, Morrison PJ, Nance M, Ross CA, Margolis RL, Rosenblatt A, Squitieri F, Frati L, Gómez-Tortosa E, García CA, Suchowersky O, Klimek ML, Trent RJ, McCusker E, Novelletto A, Frontali M, Paulsen JS, Jones R, Ashizawa T, Lazzarini A, Wheeler VC, Prakash R, Xu G, Djoussé L, Mysore JS, Gillis T, Hakky M, Cupples LA, Saint-Hilaire MH, Cha JH, Hersch SM, Penney JB, Harrison MB, Perlman SL, Zanko A, Abramson RK, Lechich AJ, Duckett A, Marder K, Conneally PM, Gusella JF, MacDonald ME, Myers RH.

BMC Med Genet. 2006 Aug 17;7:71.

30.

Discovery of bioactive small-molecule inhibitor of poly adp-ribose polymerase: implications for energy-deficient cells.

Altmann SM, Muryshev A, Fossale E, Maxwell MM, Norflus FN, Fox J, Hersch SM, Young AB, MacDonald ME, Abagyan R, Kazantsev AG.

Chem Biol. 2006 Jul;13(7):765-70.

31.

Diffusion tensor imaging in presymptomatic and early Huntington's disease: Selective white matter pathology and its relationship to clinical measures.

Rosas HD, Tuch DS, Hevelone ND, Zaleta AK, Vangel M, Hersch SM, Salat DH.

Mov Disord. 2006 Sep;21(9):1317-25.

PMID:
16755582
32.

Sp1 is up-regulated in cellular and transgenic models of Huntington disease, and its reduction is neuroprotective.

Qiu Z, Norflus F, Singh B, Swindell MK, Buzescu R, Bejarano M, Chopra R, Zucker B, Benn CL, DiRocco DP, Cha JH, Ferrante RJ, Hersch SM.

J Biol Chem. 2006 Jun 16;281(24):16672-80. Epub 2006 Apr 4.

33.

Creatine in Huntington disease is safe, tolerable, bioavailable in brain and reduces serum 8OH2'dG.

Hersch SM, Gevorkian S, Marder K, Moskowitz C, Feigin A, Cox M, Como P, Zimmerman C, Lin M, Zhang L, Ulug AM, Beal MF, Matson W, Bogdanov M, Ebbel E, Zaleta A, Kaneko Y, Jenkins B, Hevelone N, Zhang H, Yu H, Schoenfeld D, Ferrante R, Rosas HD.

Neurology. 2006 Jan 24;66(2):250-2.

PMID:
16434666
34.

Chronology of behavioral symptoms and neuropathological sequela in R6/2 Huntington's disease transgenic mice.

Stack EC, Kubilus JK, Smith K, Cormier K, Del Signore SJ, Guelin E, Ryu H, Hersch SM, Ferrante RJ.

J Comp Neurol. 2005 Oct 3;490(4):354-70.

PMID:
16127709
35.

The therapeutic role of creatine in Huntington's disease.

Ryu H, Rosas HD, Hersch SM, Ferrante RJ.

Pharmacol Ther. 2005 Nov;108(2):193-207. Epub 2005 Aug 1. Review.

PMID:
16055197
36.

Genome-wide expression profiling of human blood reveals biomarkers for Huntington's disease.

Borovecki F, Lovrecic L, Zhou J, Jeong H, Then F, Rosas HD, Hersch SM, Hogarth P, Bouzou B, Jensen RV, Krainc D.

Proc Natl Acad Sci U S A. 2005 Aug 2;102(31):11023-8. Epub 2005 Jul 25.

37.
38.
39.
40.

Anti-inflammatory treatment with acetylsalicylate or rofecoxib is not neuroprotective in Huntington's disease transgenic mice.

Norflus F, Nanje A, Gutekunst CA, Shi G, Cohen J, Bejarano M, Fox J, Ferrante RJ, Hersch SM.

Neurobiol Dis. 2004 Nov;17(2):319-25.

PMID:
15474369
41.

Cystamine increases L-cysteine levels in Huntington's disease transgenic mouse brain and in a PC12 model of polyglutamine aggregation.

Fox JH, Barber DS, Singh B, Zucker B, Swindell MK, Norflus F, Buzescu R, Chopra R, Ferrante RJ, Kazantsev A, Hersch SM.

J Neurochem. 2004 Oct;91(2):413-22.

42.

10 most commonly asked questions about Huntington's disease.

Hersch SM, Rosas HD.

Neurologist. 2001 Nov;7(6):364-8. No abstract available.

PMID:
14649632
43.

Histone deacetylase inhibition by sodium butyrate chemotherapy ameliorates the neurodegenerative phenotype in Huntington's disease mice.

Ferrante RJ, Kubilus JK, Lee J, Ryu H, Beesen A, Zucker B, Smith K, Kowall NW, Ratan RR, Luthi-Carter R, Hersch SM.

J Neurosci. 2003 Oct 15;23(28):9418-27.

44.

A genome scan for modifiers of age at onset in Huntington disease: The HD MAPS study.

Li JL, Hayden MR, Almqvist EW, Brinkman RR, Durr A, Dodé C, Morrison PJ, Suchowersky O, Ross CA, Margolis RL, Rosenblatt A, Gómez-Tortosa E, Cabrero DM, Novelletto A, Frontali M, Nance M, Trent RJ, McCusker E, Jones R, Paulsen JS, Harrison M, Zanko A, Abramson RK, Russ AL, Knowlton B, Djoussé L, Mysore JS, Tariot S, Gusella MF, Wheeler VC, Atwood LD, Cupples LA, Saint-Hilaire M, Cha JH, Hersch SM, Koroshetz WJ, Gusella JF, MacDonald ME, Myers RH.

Am J Hum Genet. 2003 Sep;73(3):682-7. Epub 2003 Aug 1.

45.
46.

Creatine therapy provides neuroprotection after onset of clinical symptoms in Huntington's disease transgenic mice.

Dedeoglu A, Kubilus JK, Yang L, Ferrante KL, Hersch SM, Beal MF, Ferrante RJ.

J Neurochem. 2003 Jun;85(6):1359-67.

47.

HIP14, a novel ankyrin domain-containing protein, links huntingtin to intracellular trafficking and endocytosis.

Singaraja RR, Hadano S, Metzler M, Givan S, Wellington CL, Warby S, Yanai A, Gutekunst CA, Leavitt BR, Yi H, Fichter K, Gan L, McCutcheon K, Chopra V, Michel J, Hersch SM, Ikeda JE, Hayden MR.

Hum Mol Genet. 2002 Nov 1;11(23):2815-28.

PMID:
12393793
48.

Therapeutic effects of cystamine in a murine model of Huntington's disease.

Dedeoglu A, Kubilus JK, Jeitner TM, Matson SA, Bogdanov M, Kowall NW, Matson WR, Cooper AJ, Ratan RR, Beal MF, Hersch SM, Ferrante RJ.

J Neurosci. 2002 Oct 15;22(20):8942-50.

49.

Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease.

Wellington CL, Ellerby LM, Gutekunst CA, Rogers D, Warby S, Graham RK, Loubser O, van Raamsdonk J, Singaraja R, Yang YZ, Gafni J, Bredesen D, Hersch SM, Leavitt BR, Roy S, Nicholson DW, Hayden MR.

J Neurosci. 2002 Sep 15;22(18):7862-72.

50.

Cytochrome C and caspase-9 expression in Huntington's disease.

Kiechle T, Dedeoglu A, Kubilus J, Kowall NW, Beal MF, Friedlander RM, Hersch SM, Ferrante RJ.

Neuromolecular Med. 2002;1(3):183-95.

PMID:
12095160

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