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Items: 1 to 50 of 829

1.

Potent and sustained huntingtin lowering via AAV5 encoding miRNA preserves striatal volume and cognitive function in a humanized mouse model of Huntington disease.

Caron NS, Southwell AL, Brouwers CC, Cengio LD, Xie Y, Black HF, Anderson LM, Ko S, Zhu X, van Deventer SJ, Evers MM, Konstantinova P, Hayden MR.

Nucleic Acids Res. 2019 Nov 20. pii: gkz976. doi: 10.1093/nar/gkz976. [Epub ahead of print]

PMID:
31745548
2.

A Comprehensive Haplotype-Targeting Strategy for Allele-Specific HTT Suppression in Huntington Disease.

Kay C, Collins JA, Caron NS, Agostinho LA, Findlay-Black H, Casal L, Sumathipala D, Dissanayake VHW, Cornejo-Olivas M, Baine F, Krause A, Greenberg JL, Paiva CLA, Squitieri F, Hayden MR.

Am J Hum Genet. 2019 Dec 5;105(6):1112-1125. doi: 10.1016/j.ajhg.2019.10.011. Epub 2019 Nov 7.

PMID:
31708117
3.
4.

In search of a genetic explanation for LDLc variability in an FH family: common SNPs and a rare mutation in MTTP explain only part of LDL variability in an FH family.

Winther M, Shpitzen S, Yaacov O, Landau J, Oren L, Foroozan-Rosenberg L, Lev Cohain N, Schurr D, Meiner V, Szalat A, Carmi S, Hayden MR, Leitersdorf E, Durst R.

J Lipid Res. 2019 Oct;60(10):1733-1740. doi: 10.1194/jlr.M092049. Epub 2019 Aug 6.

PMID:
31387896
5.

Activation of Caspase-6 Is Promoted by a Mutant Huntingtin Fragment and Blocked by an Allosteric Inhibitor Compound.

Ehrnhoefer DE, Skotte NH, Reinshagen J, Qiu X, Windshügel B, Jaishankar P, Ladha S, Petina O, Khankischpur M, Nguyen YTN, Caron NS, Razeto A, Meyer Zu Rheda M, Deng Y, Huynh KT, Wittig I, Gribbon P, Renslo AR, Geffken D, Gul S, Hayden MR.

Cell Chem Biol. 2019 Sep 19;26(9):1295-1305.e6. doi: 10.1016/j.chembiol.2019.07.001. Epub 2019 Jul 25.

PMID:
31353319
6.

Impairment and Restoration of Homeostatic Plasticity in Cultured Cortical Neurons From a Mouse Model of Huntington Disease.

Smith-Dijak AI, Nassrallah WB, Zhang LYJ, Geva M, Hayden MR, Raymond LA.

Front Cell Neurosci. 2019 May 16;13:209. doi: 10.3389/fncel.2019.00209. eCollection 2019.

7.

Pridopidine protects neurons from mutant-huntingtin toxicity via the sigma-1 receptor.

Eddings CR, Arbez N, Akimov S, Geva M, Hayden MR, Ross CA.

Neurobiol Dis. 2019 Sep;129:118-129. doi: 10.1016/j.nbd.2019.05.009. Epub 2019 May 17.

PMID:
31108174
8.

Length of Uninterrupted CAG, Independent of Polyglutamine Size, Results in Increased Somatic Instability, Hastening Onset of Huntington Disease.

Wright GEB, Collins JA, Kay C, McDonald C, Dolzhenko E, Xia Q, Bečanović K, Drögemöller BI, Semaka A, Nguyen CM, Trost B, Richards F, Bijlsma EK, Squitieri F, Ross CJD, Scherer SW, Eberle MA, Yuen RKC, Hayden MR.

Am J Hum Genet. 2019 Jun 6;104(6):1116-1126. doi: 10.1016/j.ajhg.2019.04.007. Epub 2019 May 16.

9.

Intrinsic mutant HTT-mediated defects in oligodendroglia cause myelination deficits and behavioral abnormalities in Huntington disease.

Ferrari Bardile C, Garcia-Miralles M, Caron NS, Rayan NA, Langley SR, Harmston N, Rondelli AM, Teo RTY, Waltl S, Anderson LM, Bae HG, Jung S, Williams A, Prabhakar S, Petretto E, Hayden MR, Pouladi MA.

Proc Natl Acad Sci U S A. 2019 May 7;116(19):9622-9627. doi: 10.1073/pnas.1818042116. Epub 2019 Apr 23.

11.

The combination of a neprilysin inhibitor (sacubitril) and angiotensin-II receptor blocker (valsartan) attenuates glomerular and tubular injury in the Zucker Obese rat.

Habibi J, Aroor AR, Das NA, Manrique-Acevedo CM, Johnson MS, Hayden MR, Nistala R, Wiedmeyer C, Chandrasekar B, DeMarco VG.

Cardiovasc Diabetol. 2019 Mar 25;18(1):40. doi: 10.1186/s12933-019-0847-8.

12.

Empagliflozin Ameliorates Type 2 Diabetes-Induced Ultrastructural Remodeling of the Neurovascular Unit and Neuroglia in the Female db/db Mouse.

Hayden MR, Grant DG, Aroor AR, DeMarco VG.

Brain Sci. 2019 Mar 7;9(3). pii: E57. doi: 10.3390/brainsci9030057.

13.

Altered Regulation of Striatal Neuronal N-Methyl-D-Aspartate Receptor Trafficking by Palmitoylation in Huntington Disease Mouse Model.

Kang R, Wang L, Sanders SS, Zuo K, Hayden MR, Raymond LA.

Front Synaptic Neurosci. 2019 Feb 21;11:3. doi: 10.3389/fnsyn.2019.00003. eCollection 2019.

14.

Pridopidine Induces Functional Neurorestoration Via the Sigma-1 Receptor in a Mouse Model of Parkinson's Disease.

Francardo V, Geva M, Bez F, Denis Q, Steiner L, Hayden MR, Cenci MA.

Neurotherapeutics. 2019 Apr;16(2):465-479. doi: 10.1007/s13311-018-00699-9.

15.

Pridopidine, a clinic-ready compound, reduces 3,4-dihydroxyphenylalanine-induced dyskinesia in Parkinsonian macaques.

Johnston TH, Geva M, Steiner L, Orbach A, Papapetropoulos S, Savola JM, Reynolds IJ, Ravenscroft P, Hill M, Fox SH, Brotchie JM, Laufer R, Hayden MR.

Mov Disord. 2019 May;34(5):708-716. doi: 10.1002/mds.27565. Epub 2018 Dec 21.

PMID:
30575996
16.

Identification of a novel caspase cleavage site in huntingtin that regulates mutant huntingtin clearance.

Martin DDO, Schmidt ME, Nguyen YT, Lazic N, Hayden MR.

FASEB J. 2019 Mar;33(3):3190-3197. doi: 10.1096/fj.201701510RRR. Epub 2018 Nov 13.

PMID:
30423259
17.

Laquinimod Treatment Improves Myelination Deficits at the Transcriptional and Ultrastructural Levels in the YAC128 Mouse Model of Huntington Disease.

Garcia-Miralles M, Yusof NABM, Tan JY, Radulescu CI, Sidik H, Tan LJ, Belinson H, Zach N, Hayden MR, Pouladi MA.

Mol Neurobiol. 2019 Jun;56(6):4464-4478. doi: 10.1007/s12035-018-1393-1. Epub 2018 Oct 17.

PMID:
30334188
18.

Huntingtin suppression restores cognitive function in a mouse model of Huntington's disease.

Southwell AL, Kordasiewicz HB, Langbehn D, Skotte NH, Parsons MP, Villanueva EB, Caron NS, Østergaard ME, Anderson LM, Xie Y, Cengio LD, Findlay-Black H, Doty CN, Fitsimmons B, Swayze EE, Seth PP, Raymond LA, Frank Bennett C, Hayden MR.

Sci Transl Med. 2018 Oct 3;10(461). pii: eaar3959. doi: 10.1126/scitranslmed.aar3959.

PMID:
30282695
19.

Therapeutic approaches to Huntington disease: from the bench to the clinic.

Caron NS, Dorsey ER, Hayden MR.

Nat Rev Drug Discov. 2018 Oct;17(10):729-750. doi: 10.1038/nrd.2018.133. Epub 2018 Sep 21. Review.

PMID:
30237454
20.

Physicochemical, biological, functional and toxicological characterization of the European follow-on glatiramer acetate product as compared with Copaxone.

Melamed-Gal S, Loupe P, Timan B, Weinstein V, Kolitz S, Zhang J, Funt J, Komlosh A, Ashkenazi N, Bar-Ilan O, Konya A, Beriozkin O, Laifenfeld D, Hasson T, Krispin R, Molotsky T, Papir G, Sulimani L, Zeskind B, Liu P, Nock S, Hayden MR, Gilbert A, Grossman I.

eNeurologicalSci. 2018 May 30;12:19-30. doi: 10.1016/j.ensci.2018.05.006. eCollection 2018 Sep.

21.

Glycemic control by the SGLT2 inhibitor empagliflozin decreases aortic stiffness, renal resistivity index and kidney injury.

Aroor AR, Das NA, Carpenter AJ, Habibi J, Jia G, Ramirez-Perez FI, Martinez-Lemus L, Manrique-Acevedo CM, Hayden MR, Duta C, Nistala R, Mayoux E, Padilla J, Chandrasekar B, DeMarco VG.

Cardiovasc Diabetol. 2018 Jul 30;17(1):108. doi: 10.1186/s12933-018-0750-8.

22.

Pharmacogenomics of Vincristine-Induced Peripheral Neuropathy Implicates Pharmacokinetic and Inherited Neuropathy Genes.

Wright GEB, Amstutz U, Drögemöller BI, Shih J, Rassekh SR, Hayden MR, Carleton BC, Ross CJD; Canadian Pharmacogenomics Network for Drug Safety Consortium.

Clin Pharmacol Ther. 2019 Feb;105(2):402-410. doi: 10.1002/cpt.1179. Epub 2018 Aug 17.

23.

Huntington Disease.

Caron NS, Wright GEB, Hayden MR.

In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2019.
1998 Oct 23 [updated 2018 Jul 5].

24.

Altering cortical input unmasks synaptic phenotypes in the YAC128 cortico-striatal co-culture model of Huntington disease.

Schmidt ME, Buren C, Mackay JP, Cheung D, Dal Cengio L, Raymond LA, Hayden MR.

BMC Biol. 2018 Jun 27;16(1):58. doi: 10.1186/s12915-018-0526-3.

25.

A human huntingtin SNP alters post-translational modification and pathogenic proteolysis of the protein causing Huntington disease.

Martin DDO, Kay C, Collins JA, Nguyen YT, Slama RA, Hayden MR.

Sci Rep. 2018 May 25;8(1):8096. doi: 10.1038/s41598-018-25903-w.

26.

Large-scale transcriptomic analysis reveals that pridopidine reverses aberrant gene expression and activates neuroprotective pathways in the YAC128 HD mouse.

Kusko R, Dreymann J, Ross J, Cha Y, Escalante-Chong R, Garcia-Miralles M, Tan LJ, Burczynski ME, Zeskind B, Laifenfeld D, Pouladi M, Geva M, Grossman I, Hayden MR.

Mol Neurodegener. 2018 May 21;13(1):25. doi: 10.1186/s13024-018-0259-3.

27.

A whole brain longitudinal study in the YAC128 mouse model of Huntington's disease shows distinct trajectories of neurochemical, structural connectivity and volumetric changes.

Petrella LI, Castelhano JM, Ribeiro M, Sereno JV, Gonçalves SI, Laço MN, Hayden MR, Rego AC, Castelo-Branco M.

Hum Mol Genet. 2018 Jun 15;27(12):2125-2137. doi: 10.1093/hmg/ddy119.

PMID:
29668904
28.

Constitutive ablation of caspase-6 reduces the inflammatory response and behavioural changes caused by peripheral pro-inflammatory stimuli.

Ladha S, Qiu X, Casal L, Caron NS, Ehrnhoefer DE, Hayden MR.

Cell Death Discov. 2018 Mar 12;4:40. doi: 10.1038/s41420-018-0043-8. eCollection 2018.

29.

Preventing mutant huntingtin proteolysis and intermittent fasting promote autophagy in models of Huntington disease.

Ehrnhoefer DE, Martin DDO, Schmidt ME, Qiu X, Ladha S, Caron NS, Skotte NH, Nguyen YTN, Vaid K, Southwell AL, Engemann S, Franciosi S, Hayden MR.

Acta Neuropathol Commun. 2018 Mar 6;6(1):16. doi: 10.1186/s40478-018-0518-0.

30.

Therapeutic modulation of the bile acid pool by Cyp8b1 knockdown protects against nonalcoholic fatty liver disease in mice.

Chevre R, Trigueros-Motos L, Castaño D, Chua T, Corlianò M, Patankar JV, Sng L, Sim L, Juin TL, Carissimo G, Ng LFP, Yi CNJ, Eliathamby CC, Groen AK, Hayden MR, Singaraja RR.

FASEB J. 2018 Jul;32(7):3792-3802. doi: 10.1096/fj.201701084RR. Epub 2018 Feb 26.

PMID:
29481310
31.

The molecular epidemiology of Huntington disease is related to intermediate allele frequency and haplotype in the general population.

Kay C, Collins JA, Wright GEB, Baine F, Miedzybrodzka Z, Aminkeng F, Semaka AJ, McDonald C, Davidson M, Madore SJ, Gordon ES, Gerry NP, Cornejo-Olivas M, Squitieri F, Tishkoff S, Greenberg JL, Krause A, Hayden MR.

Am J Med Genet B Neuropsychiatr Genet. 2018 Apr;177(3):346-357. doi: 10.1002/ajmg.b.32618. Epub 2018 Feb 20.

PMID:
29460498
32.

Further Investigation of the Role of ACYP2 and WFS1 Pharmacogenomic Variants in the Development of Cisplatin-Induced Ototoxicity in Testicular Cancer Patients.

Drögemöller BI, Brooks B, Critchley C, Monzon JG, Wright GEB, Liu G, Renouf DJ, Kollmannsberger CK, Bedard PL, Hayden MR, Gelmon KA, Carleton BC, Ross CJD.

Clin Cancer Res. 2018 Apr 15;24(8):1866-1871. doi: 10.1158/1078-0432.CCR-17-2810. Epub 2018 Jan 22.

33.

Early pridopidine treatment improves behavioral and transcriptional deficits in YAC128 Huntington disease mice.

Garcia-Miralles M, Geva M, Tan JY, Yusof NABM, Cha Y, Kusko R, Tan LJ, Xu X, Grossman I, Orbach A, Hayden MR, Pouladi MA.

JCI Insight. 2017 Dec 7;2(23). pii: 95665. doi: 10.1172/jci.insight.95665.

34.

Compositional differences between Copaxone and Glatopa are reflected in altered immunomodulation ex vivo in a mouse model.

Grossman I, Kolitz S, Komlosh A, Zeskind B, Weinstein V, Laifenfeld D, Gilbert A, Bar-Ilan O, Fowler KD, Hasson T, Konya A, Wells-Knecht K, Loupe P, Melamed-Gal S, Molotsky T, Krispin R, Papir G, Sahly Y, Hayden MR.

Ann N Y Acad Sci. 2017 Nov;1407(1):75-89. doi: 10.1111/nyas.13547.

PMID:
29168242
35.

HACE1 is essential for astrocyte mitochondrial function and influences Huntington disease phenotypes in vivo.

Ehrnhoefer DE, Southwell AL, Sivasubramanian M, Qiu X, Villanueva EB, Xie Y, Waltl S, Anderson L, Fazeli A, Casal L, Felczak B, Tsang M, Hayden MR.

Hum Mol Genet. 2018 Jan 15;27(2):239-253. doi: 10.1093/hmg/ddx394.

36.

Genetic ablation of Cyp8b1 preserves host metabolic function by repressing steatohepatitis and altering gut microbiota composition.

Patankar JV, Wong CK, Morampudi V, Gibson WT, Vallance B, Ioannou GN, Hayden MR.

Am J Physiol Endocrinol Metab. 2018 May 1;314(5):E418-E432. doi: 10.1152/ajpendo.00172.2017. Epub 2017 Oct 24.

37.

Epidemiology of Huntington disease.

Kay C, Hayden MR, Leavitt BR.

Handb Clin Neurol. 2017;144:31-46. doi: 10.1016/B978-0-12-801893-4.00003-1. Review.

PMID:
28947124
38.

Characterization of subventricular zone-derived progenitor cells from mild and late symptomatic YAC128 mouse model of Huntington's disease.

Silva AC, Ferreira IL, Hayden MR, Ferreiro E, Rego AC.

Biochim Biophys Acta Mol Basis Dis. 2018 Jan;1864(1):34-44. doi: 10.1016/j.bbadis.2017.09.009. Epub 2017 Sep 20.

39.

ABCA8 Regulates Cholesterol Efflux and High-Density Lipoprotein Cholesterol Levels.

Trigueros-Motos L, van Capelleveen JC, Torta F, Castaño D, Zhang LH, Chai EC, Kang M, Dimova LG, Schimmel AWM, Tietjen I, Radomski C, Tan LJ, Thiam CH, Narayanaswamy P, Wu DH, Dorninger F, Yakala GK, Barhdadi A, Angeli V, Dubé MP, Berger J, Dallinga-Thie GM, Tietge UJF, Wenk MR, Hayden MR, Hovingh GK, Singaraja RR.

Arterioscler Thromb Vasc Biol. 2017 Nov;37(11):2147-2155. doi: 10.1161/ATVBAHA.117.309574. Epub 2017 Sep 7.

PMID:
28882873
40.

A pharmacogenetic signature of high response to Copaxone in late-phase clinical-trial cohorts of multiple sclerosis.

Ross CJ, Towfic F, Shankar J, Laifenfeld D, Thoma M, Davis M, Weiner B, Kusko R, Zeskind B, Knappertz V, Grossman I, Hayden MR.

Genome Med. 2017 May 31;9(1):50. doi: 10.1186/s13073-017-0436-y.

41.

Xanthine oxidase inhibition protects against Western diet-induced aortic stiffness and impaired vasorelaxation in female mice.

Lastra G, Manrique C, Jia G, Aroor AR, Hayden MR, Barron BJ, Niles B, Padilla J, Sowers JR.

Am J Physiol Regul Integr Comp Physiol. 2017 Aug 1;313(2):R67-R77. doi: 10.1152/ajpregu.00483.2016. Epub 2017 May 24.

42.

Dipeptidyl peptidase-4 (DPP-4) inhibition with linagliptin reduces western diet-induced myocardial TRAF3IP2 expression, inflammation and fibrosis in female mice.

Aroor AR, Habibi J, Kandikattu HK, Garro-Kacher M, Barron B, Chen D, Hayden MR, Whaley-Connell A, Bender SB, Klein T, Padilla J, Sowers JR, Chandrasekar B, DeMarco VG.

Cardiovasc Diabetol. 2017 May 5;16(1):61. doi: 10.1186/s12933-017-0544-4.

43.

Association Between SLC16A5 Genetic Variation and Cisplatin-Induced Ototoxic Effects in Adult Patients With Testicular Cancer.

Drögemöller BI, Monzon JG, Bhavsar AP, Borrie AE, Brooks B, Wright GEB, Liu G, Renouf DJ, Kollmannsberger CK, Bedard PL, Aminkeng F, Amstutz U, Hildebrand CA, Gunaretnam EP, Critchley C, Chen Z, Brunham LR, Hayden MR, Ross CJD, Gelmon KA, Carleton BC.

JAMA Oncol. 2017 Nov 1;3(11):1558-1562. doi: 10.1001/jamaoncol.2017.0502.

44.

Neurodegeneration: Role of repeats in protein clearance.

Martin DDO, Hayden MR.

Nature. 2017 May 4;545(7652):33-34. doi: 10.1038/nature22489. Epub 2017 Apr 26. No abstract available.

PMID:
28445458
45.

Pharmacogenomic screening for anthracycline-induced cardiotoxicity in childhood cancer.

Aminkeng F, Ross CJD, Rassekh SR, Rieder MJ, Bhavsar AP, Sanatani S, Bernstein D, Hayden MR, Amstutz U, Carleton BC.

Br J Clin Pharmacol. 2017 May;83(5):1143-1145. doi: 10.1111/bcp.13218. Epub 2017 Mar 19. No abstract available.

46.

Histone Deacetylase Inhibitors Protect Against Pyruvate Dehydrogenase Dysfunction in Huntington's Disease.

Naia L, Cunha-Oliveira T, Rodrigues J, Rosenstock TR, Oliveira A, Ribeiro M, Carmo C, Oliveira-Sousa SI, Duarte AI, Hayden MR, Rego AC.

J Neurosci. 2017 Mar 8;37(10):2776-2794. doi: 10.1523/JNEUROSCI.2006-14.2016. Epub 2017 Jan 25.

47.

A novel humanized mouse model of Huntington disease for preclinical development of therapeutics targeting mutant huntingtin alleles.

Southwell AL, Skotte NH, Villanueva EB, Østergaard ME, Gu X, Kordasiewicz HB, Kay C, Cheung D, Xie Y, Waltl S, Dal Cengio L, Findlay-Black H, Doty CN, Petoukhov E, Iworima D, Slama R, Ooi J, Pouladi MA, Yang XW, Swayze EE, Seth PP, Hayden MR.

Hum Mol Genet. 2017 Mar 15;26(6):1115-1132. doi: 10.1093/hmg/ddx021.

PMID:
28104789
48.

Author response: Huntington disease reduced penetrance alleles occur at high frequency in the general population.

Hayden MR, Kay C.

Neurology. 2017 Jan 17;88(3):334-335. doi: 10.1212/WNL.0000000000003528. No abstract available.

PMID:
28093515
49.

Sodium glucose transporter 2 (SGLT2) inhibition with empagliflozin improves cardiac diastolic function in a female rodent model of diabetes.

Habibi J, Aroor AR, Sowers JR, Jia G, Hayden MR, Garro M, Barron B, Mayoux E, Rector RS, Whaley-Connell A, DeMarco VG.

Cardiovasc Diabetol. 2017 Jan 13;16(1):9. doi: 10.1186/s12933-016-0489-z.

50.

The targetable A1 Huntington disease haplotype has distinct Amerindian and European origins in Latin America.

Kay C, Tirado-Hurtado I, Cornejo-Olivas M, Collins JA, Wright G, Inca-Martinez M, Veliz-Otani D, Ketelaar ME, Slama RA, Ross CJ, Mazzetti P, Hayden MR.

Eur J Hum Genet. 2017 Feb;25(3):332-340. doi: 10.1038/ejhg.2016.169. Epub 2016 Dec 21.

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