CHDI
2018
Authors
Title
Journal
- Beckmann H, | Bohlen S, | Saft C, | Hoffmann R, | Gerss J, | Muratori L, | Ringelstein EB, | Landwehrmeyer GB, | Reilmann R |Objective assessment of gait and posture in premanifest and manifest Huntington disease - A multi-center studyGait Posture451-45762():20180417PMID:29660633Lai JS, | Goodnight S, | Downing NR, | Ready RE, | Paulsen JS, | Kratz AL, | Stout JC, | McCormack MK, | Cella D, | Ross C, | Russell J, | Carlozzi NE |Evaluating cognition in individuals with Huntington disease: Neuro-QoL cognitive functioning measuresQual Life Res811-82227(3):20180330PMID:29222609PMCID: 5845825Ament SA, | Pearl JR, | Cantle JP, | Bragg RM, | Skene PJ, | Coffey SR, | Bergey DE, | Wheeler VC, | MacDonald ME, | Baliga NS, | Rosinski J, | Hood LE, | Carroll JB, | Price ND |Transcriptional regulatory networks underlying gene expression changes in Huntington's diseaseMol Syst Biole743514(3):20180326PMID:29581148PMCID: 5868199Bresciani A, | Spiezia MC, | Boggio R, | Cariulo C, | Nordheim A, | Altobelli R, | Kuhlbrodt K, | Dominguez C, | Munoz-Sanjuan I, | Wityak J, | Fodale V, | Marchionini DM, | Weiss A |Quantifying autophagy using novel LC3B and p62 TR-FRET assaysPLoS Onee019442313(3):20180319PMID:29554128PMCID: 5858923Ouk K, | Aungier J, | Cuesta M, | Morton AJ |Chronic paroxetine treatment prevents disruption of methamphetamine-sensitive circadian oscillator in a transgenic mouse model of Huntington's diseaseNeuropharmacology337-350131():20180315PMID:29274752Minkova L, | Gregory S, | Scahill RI, | Abdulkadir A, | Kaller CP, | Peter J, | Long JD, | Stout JC, | Reilmann R, | Roos RA, | Durr A, | Leavitt BR, | Tabrizi SJ, | Klöppel S, | TRACK-HD Investigators |Cross-sectional and longitudinal voxel-based grey matter asymmetries in Huntington's diseaseNeuroimage Clin312-32417():20180313PMID:29527479PMCID: 5842644Espinoza FA, | Turner JA, | Vergara VM, | Miller RL, | Mennigen E, | Liu J, | Misiura MB, | Ciarochi J, | Johnson HJ, | Long JD, | Bockholt HJ, | Magnota VA, | Paulsen JS, | Calhoun VD |Whole-brain connectivity in a large study of Huntington's disease gene mutation carriers and healthy controlsBrain Connect():20180308PMID:29291624Lee CYD, | Daggett A, | Gu X, | Jiang LL, | Langfelder P, | Li X, | Wang N, | Zhao Y, | Park CS, | Cooper Y, | Ferando I, | Mody I, | Coppola G, | Xu H, | Yang XW |Elevated TREM2 gene dosage reprograms microglia responsivity and ameliorates pathological phenotypes in Alzheimer's disease modelsNeuron1032-104897(5):20180307PMID:29518357Guo Q, | Bin Huang, | Cheng J, | Seefelder M, | Engler T, | Pfeifer G, | Oeckl P, | Otto M, | Moser F, | Maurer M, | Pautsch A, | Baumeister W, | Fernández-Busnadiego R, | Kochanek S |The cryo-electron microscopy structure of huntingtinNature117-120555(7694):20180301PMID:29466333DOI: 10.1038/nature25502Odish OFF, | Reijntjes RHAM, | van den Bogaard SJA, | Roos RAC, | Leemans A |Progressive microstructural changes of the occipital cortex in Huntington's diseaseBrain Imaging Behav():20180228PMID:29492750Kielar C, | Morton AJ |Early neurodegeneration in R6/2 mice carrying the Huntington's disease mutation with a super-expanded CAG repeat, despite normal lifespanJ Huntingtons Dis61-767(1):20180227PMID:29480204DOI: 10.3233/JHD-170265Kovalenko M, | Milnerwood A, | Giordano J, | St Claire J, | Guide JR, | Stromberg M, | Gillis T, | Sapp E, | DiFiglia M, | MacDonald ME, | Carroll JB, | Lee JM, | Tappan S, | Raymond L, | Wheeler VC |HttQ111/+ Huntington's disease knock-in mice exhibit brain region-specific morphological changes and synaptic dysfunctionJ Huntingtons Dis17-337(1):20180224PMID:29480209DOI: 10.3233/JHD-170282Pfister EL, DiNardo N, Mondo E, Borel F, Conroy F, Fraser C, | Gernoux G, | Han X, | Hu D, | Johnson E, | Kennington L, | Liu P, | Reid SJ, | Sapp E, | Vodicka P, | Kuchel T, | Morton AJ, | Howland D, | Moser R, | Sena-Esteves M, | Gao G, | Mueller C, | DiFiglia M, | Aronin N |Artificial miRNAs reduce Human Mutant Huntingtin throughout the striatum in a transgenic sheep model of Huntington's diseaseHum Gene Ther():20180223PMID:29207890DOI: 10.1089/hum.2017.199Veldman MB, | Yang XW |Molecular insights into cortico-striatal miscommunications in Huntington's diseaseCurr Opin Neurobiol79-8948():20180222PMID:29125980Rebec GV |Corticostriatal network dysfunction in huntington's disease: Deficits in neural processing, glutamate transport, and ascorbate releaseCNS Neurosci Ther281-29124(4):20180221PMID:29464896DOI: 10.1111/cns.12828Sampaio C, | Levey J, | Klitzman R |Predictive testing and clinical trials in Huntington's disease: An ethical analysisMov Disord243-24733(2):20180220PMID:29205501DOI: 10.1002/mds.27247Mestre TA, | Bachoud-Lévi AC, | Marinus J, | Stout JC, | Paulsen JS, | Como P, | Duff K, | Sampaio C, | Goetz CG, | Cubo E, | Stebbins GT, | Martinez-Martin P, | Members of the MDS Committee on Rating Scales Development |Rating scales for cognition in Huntington's disease: Critique and recommendationsMov Disord187-19533(2):20180219PMID:29278291DOI: 10.1002/mds.27227Hassler MR, | Turanov AA, | Alterman JF, | Haraszti RA, | Coles AH, | Osborn MF, | Echeverria D, | Nikan M, | Salomon WE, | Roux L, | Godinho BMDC, | Davis SM, | Morrissey DV, | Zamore PD, | Karumanchi SA, | Moore MJ, | Aronin N, | Khvorova A |Comparison of partially and fully chemically-modified siRNA in conjugate-mediated delivery in vivoNucleic Acids Res1-12():20180208PMID:29432571DOI: 10.1093/nar/gky037Arnerić SP, | Kern VD, | Stephenson DT |Regulatory-accepted drug development tools are needed to accelerate innovative CNS disease treatmentsBiochem Pharmacol291-306151():20180130PMID:29410157Ruzo A, | Croft GF, | Metzger JJ, | Galgoczi S, | Gerber LJ, | Pellegrini C, | Wang H Jr, | Fenner M, | Tse S, | Marks A, | Nchako C, | Brivanlou AH |Chromosomal instability during neurogenesis in Huntington's diseaseDevelopmentdev156844145(2):20180129PMID:29378824DOI: 10.1242/dev.156844Johnson EB, | Byrne LM, | Gregory S, | Rodrigues FB, | Blennow K, | Durr A, | Leavitt BR, | Roos RA, | Zetterberg H, | Tabrizi SJ, | Scahill RI, | Wild EJ, | TRACK-HD Study Group |Neurofilament light protein in blood predicts regional atrophy in Huntington diseaseNeurology():20180124PMID:29367444Casula EP, | Mayer IMS, | Desikan M, | Tabrizi SJ, | Rothwell JC, | Orth M |Motor cortex synchronization influences the rhythm of motor performance in premanifest Huntington's diseaseMov Disord440-44833(3):20180122PMID:29356133DOI: 10.1002/mds.27285Sepers MD, | Smith-Dijak A, | LeDue J, | Kolodziejczyk K, | Mackie K, | Raymond LA |Endocannabinoid-specific impairment in synaptic plasticity in striatum of Huntington's disease mouse modelJ Neurosci544-55438(3):20180117PMID:29192125Langfelder P, | Gao F, | Wang N, | Howland D, | Kwak S, | Vogt TF, | Aaronson JS, | Rosinski J, | Coppola G, | Horvath S, | Yang XW |MicroRNA signatures of endogenous Huntingtin CAG repeat expansion in micePLoS Onee019055013(1):20180111PMID:29324753Carlozzi NE, | Hahn EA, | Frank SA, | Perlmutter JS, | Downing ND, | McCormack MK, | Barton S, | Nance MA, | Schilling SG, | HDQLIFE Site Investigators and Coordinators |A new measure for end of life planning, preparation, and preferences in Huntington disease: HDQLIFE end of life planningJ Neurol98-107265(1):20180110PMID:29143208PMCID: 5762414Sbodio JI, | Snyder SH, | Paul BD |Golgi stress response reprograms cysteine metabolism to confer cytoprotection in Huntington's diseaseProc Natl Acad Sci USA():20180109PMID:29317536Conforti P, | Besusso D, | Bocchi VD, | Faedo A, | Cesana E, | Rossetti G, | Ranzani V, | Svendsen CN, | Thompson LM, | Toselli M, | Biella G, | Pagani M, | Cattaneo E |Faulty neuronal determination and cell polarization are reverted by modulating HD early phenotypesProc Natl Acad Sci USAE762-E771115(4):20180108PMID:29311338Wang HB, | Loh DH, | Whittaker DS, | Cutler T, | Howland D, | Colwell CS |Time-restricted feeding improves circadian dysfunction as well as motor symptoms in the Q175 mouse model of Huntington's diseaseeNeuro5(1):20180103PMID:29302618PMCID: 5752678
2017
AuthorsTitleJournal- Al-Ramahi I, | Panapakkam Giridharan SS, | Chen YC, | Patnaik S, | Safren N, | Hasegawa J, | de Haro M, | Wagner Gee AK, | Titus SA, | Jeong H, | Clarke J, | Krainc D, | Zheng W, | Irvine RF, | Barmada S, | Ferrer M, | Southall N, | Weisman LS, | Botas J, | Marugan JJ |Inhibition of PIP4Kγ ameliorates the pathological effects of mutant huntingtin proteinelifee291236():20171226PMID:29256861DOI: 10.7554/eLife.29123PMCID: 5743427Hensman Moss DJ, | Robertson N, | Farmer R, | Scahill RI, | Haider S, | Tessari MA, | Flynn G, | Fischer DF, | Wild EJ, | Macdonald D, | Tabrizi SJ |Quantification of huntingtin protein species in Huntington's disease patient leukocytes using optimised electrochemiluminescence immunoassaysPLoS Onee018989112(12):20171222PMID:29272284Fodale V, | Boggio R, | Daldin M, | Cariulo C, | Spiezia MC, | Byrne LM, | Leavitt BR, | Wild EJ, | Macdonald D, | Weiss A, | Bresciani A |Validation of ultrasensitive mutant Huntingtin detection in human cerebrospinal fluid by single molecule counting immunoassayJ Huntingtons Dis349-3616(4):20171212PMID:29125493DOI: 10.3233/JHD-170269Schobel SA, | Palermo G, | Auinger P, | Long JD, | Ma S, | Khwaja OS, | Trundell D, | Cudkowicz M, | Hersch S, | Sampaio C, | Dorsey ER, | Leavitt BR, | Kieburtz KD, | Sevigny JJ, | Langbehn DR, | Tabrizi SJ, | TRACK-HD, | COHORT, | CARE-HD, | and 2CARE Huntington Study Group Investigators |Motor, cognitive, and functional declines contribute to a single progressive factor in early HDNeurology2495-250289(24):20171212PMID:29142089PMCID: 5729794Dickey AS, | Sanchez DN, | Arreola M, | Sampat KR, | Fan W, | Arbez N, | Akimov S, | Van Kanegan MJ, | Ohnishi K, | Gilmore-Hall SK, | Flores AL, | Nguyen JM, | Lomas N, | Hsu CL, | Lo DC, | Ross CA, | Masliah E, | Evans RM, | La Spada AR |PPARδ activation by bexarotene promotes neuroprotection by restoring bioenergetic and quality control homeostasisSci Transl Medeaal23329(419):20171206PMID:29212711PMCID: 5748332Arbez N, | Ratovitski T, | Roby E, | Chighladze E, | Stewart JC, | Ren M, | Wang X, | Lavery DJ, | Ross CA|Post-translational modifications clustering within proteolytic domains decrease mutant huntingtin toxicityJ Biol Chem19238-19249292(47):20171124PMID:28972180Friedman A, | Homma D, | Bloem B, | Gibb LG, | Amemori KI, | Hu D, | Delcasso S, | Truong TF, | Yang J, | Hood AS, | Mikofalvy KA, | Beck DW, | Nguyen N, | Nelson ED, | Toro Arana SE, | Vorder Bruegge RH, | Goosens KA, | Graybiel AM |Chronic stress alters striosome-circuit dynamics, leading to aberrant decision-makingCell1191-1205171(5):20171116PMID:29149606PMCID: 5734095Chao MJ, | Gillis T, | Atwal RS, | Mysore JS, | Arjomand J, | Harold D, | Holmans P, | Jones L, | Orth M, | Myers RH, | Kwak S, | Wheeler VC, | MacDonald ME, | Gusella JF, | Lee JM |Haplotype-based stratification of Huntington's diseaseEur J Hum Genet1202-120925(11):20171101PMID:28832564Brown KE, | Lohse KR, | Mayer IMS, | Strigaro G, | Desikan M, | Casula EP, | Meunier S, | Popa T, | Lamy JC, | Odish O, | Leavitt BR, | Durr A, | Roos RAC, | Tabrizi SJ, | Rothwell JC, | Boyd LA, | Orth M |The reliability of commonly used electrophysiology measuresBrain Stimul1102-111110(6):20171101PMID:28807846Wu D, | Faria AV, | Younes L, | Mori S, | Brown T, | Johnson H, | Paulsen JS, | Ross CA, | Miller MI, | PREDICT-HD Investigators |Coordinators of the Huntington Study Group |Mapping the order and pattern of brain structural MRI changes using change-point analysis in premanifest Huntington's diseaseHum Brain Mapp5035-505038(10):20171031PMID:28657159DOI: 10.1002/hbm.23713Arnerić SP, | Cedarbaum JM, | Khozin S, | Papapetropoulos S, | Hill DL, | Ropacki M, | Rhodes J, | Dacks PA, | Hudson LD, | Gordon MF, | Kern VD, | Romero K, | Vradenburg G, | Au R, | Karlin DR, | Facheris MF, | Fitzer-Attas CJ, | Vitolo OV, | Wang J, | Miller BM, | Kaye JA |Biometric monitoring devices for assessing end points in clinical trials: developing an ecosystemNat Rev Drug Discov16(10):20171031PMID:28935908DOI: 10.1038/nrd.2017.153Wild EJ, | Tabrizi SJ |Therapies targeting DNA and RNA in Huntington's diseaseLancet Neurol837-84716(10):20171031PMID:28920889Whittaker DS, | Wang HB, | Loh DH, | Cachope R, | Colwell CS |Possible use of a H3R antagonist for the management of nonmotor symptoms in the Q175 mouse model of Huntington's diseasePharmacol Res Perspecte003445(5):20171030PMID:28971617DOI: 10.1002/prp2.344PMCID: 5625154Bondulich 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 |Myostatin inhibition prevents skeletal muscle pathophysiology in Huntington's disease miceSci Rep142757(1):20171027PMID:29079832Warner JB 4th, | Ruff KM, | Tan PS, | Lemke EA, | Pappu RV, | Lashuel HA |Monomeric Huntingtin Exon 1 has similar overall structural features for wild-type and pathological polyglutamine lengthsJ Am Chem Soc14456-14469139(41):20171018PMID:28937758DOI: 10.1021/jacs.7b06659Schuldenzucker V, | Schubert R, | Muratori LM, | Freisfeld F, | Rieke L, | Matheis T, | Schramke S, | Motlik J, | Kemper N, | Radespiel U, | Reilmann R |Behavioral testing of minipigs transgenic for the Huntington gene - A three-year observational studyPLoS Onee018597012(10):20171009PMID:29016656PMCID: 5633197Bresciani A, | Missineo A, | Gallo M, | Cerretani M, | Fezzardi P, | Tomei L, | Cicero DO, | Altamura S, | Santoprete A, | Ingenito R, | Bianchi E, | Pacifici R, | Dominguez C, | Munoz-Sanjuan I, | Harper S, | Toledo-Sherman L, | Park LC |Nuclear factor (erythroid-derived 2)-like 2 (NRF2) drug discovery: Biochemical toolbox to develop NRF2 activators by reversible binding of Kelch-like ECH-associated protein 1 (KEAP1)Arch Biochem Biophys31-41631():20171001PMID:28801166Lee JM, | Chao MJ, | Harold D, | Abu Elneel K, | Gillis T, | Holmans P, | Jones L, | Orth M, | Myers RH, | Kwak S, | Wheeler VC, | MacDonald ME, | Gusella JF |A modifier of Huntington's disease onset at the MLH1 locusHum Mol Genet3859-386726(19):20171001PMID:28934397DOI: 10.1093/hmg/ddx286Long JD, | Mills JA, | Leavitt BR, | Durr A, | Roos RA, | Stout JC, | Reilmann R, | Landwehrmeyer B, | Gregory S, | Scahill RI, | Langbehn DR, | Tabrizi SJ, | Track-HD and Track-On Investigators |Survival end points for Huntington disease trials prior to a motor diagnosisJAMA Neurol():20170918PMID:28975278Frost C, | Mulick A, | Scahill RI, | Owen G, | Aylward E, | Leavitt BR, | Durr A, | Roos RAC, | Borowsky B, | Stout JC, | Reilmann R, | Langbehn DR, | Tabrizi SJ, | Sampaio C, | TRACK-HD Investigators |Design optimization for clinical trials in early-stage manifest Huntington's diseaseMov Disord():20170914PMID:28906031DOI: 10.1002/mds.27122Niccolini F, | Pagano G, | Fusar-Poli P, | Wood A, | Mrzljak L, | Sampaio C, | Politis M |Striatal molecular alterations in HD gene carriers: a systematic review and meta-analysis of PET studiesJ Neurol Neurosurg Psychiatry():20170909PMID:28889093Westervelt HJ, | Bernier RA, | Faust M, | Gover M, | Bockholt HJ, | Zschiegner R, | Long JD, | Paulsen JS, | PREDICT-HD Investigators, | Coordinators of the Huntington Study Group |Data quality assurance and control in cognitive research: Lessons learned from the PREDICT-HD studyInt J Methods Psychiatr Res1-926(3):20170901PMID:28211597DOI: 10.1002/mpr.1534Hensman Moss DJ, | Pardiñas AF, | Langbehn D, | Lo K, | Leavitt BR, | Roos R, | Durr A, | Mead S, | TRACK-HD investigators, | REGISTRY investigators, | Holmans P, | Jones L, | Tabrizi SJ |Identification of genetic variants associated with Huntington's disease progression: a genome-wide association studyLancet Neurol701-71116(9):20170901PMID:28642124van der Burg JMM, | Gardiner SL, | Ludolph AC, | Landwehrmeyer GB, | Roos RAC, | Aziz NA |Body weight is a robust predictor of clinical progression in Huntington diseaseAnn Neurol479-48382(3):20170901PMID:28779551DOI: 10.1002/ana.25007Brady ST, | Morfini GA |Regulation of motor proteins, axonal transport deficits and adult-onset neurodegenerative diseasesNeurobiol Dis273-282105():20170901PMID:28411118PMCID: 5522763Schain M, | Fazio P, | Mrzljak L, | Amini N, | Al-Tawil N, | Fitzer-Attas C, | Bronzova J, | Landwehrmeyer B, | Sampaio C, | Halldin C, | Varrone A |Revisiting the Logan plot to account for non-negligible blood volume in brain tissueEJNMMI Res7(66):20170818PMID:28822101PMCID: 5561763Ratovitski T, | O'Meally RN, | Jiang M, | Chaerkady R, | Chighladze E, | Stewart JC, | Wang X, | Arbez N, | Roby E, | Alexandris A, | Duan W, | Vijayvargia R, | Seong IS, | Lavery DJ, | Cole RN, | Ross CA |Post-Translational Modifications (PTMs), identified on endogenous Huntingtin, cluster within proteolytic domains between HEAT repeatsJ Proteome Res2692-270816(8):20170804PMID:28653853PMCID: 5560079Zheng P, | Kozloski J |Striatal network models of Huntington's disease dysfunction phenotypesFront Comput Neurosci11(70):20170727PMID:28798680PMCID: 5529396Ghosh S, | Sun Z, | Li Y, | Cheng Y, | Mohan A, | Sampaio C, | Hu J |An exploration of latent structure in observational Huntington's disease studiesAMIA Jt Summits Transl Sci Proc92-1022017():20170726PMID:28815114PMCID: 5543350Daldin M, | Fodale V, | Cariulo C, | Azzollini L, | Verani M, | Martufi P, | Spiezia MC, | Deguire SM, | Cherubini M, | Macdonald D, | Weiss A, | Bresciani A, | Vonsattel JG, | Petricca L, | Marsh JL, | Gines S, | Santimone I, | Marano M, | Lashuel HA. | Squitieri F, | Caricasole A |Polyglutamine expansion affects huntingtin conformation in multiple Huntington's disease modelsSci Reports1-137(5070):20170711PMID:28698602PMCID: 5505970Kang H, | Vázquez FX, | Zhang L, | Das P, | Toledo-Sherman L, | Luan B, | Levitt M, | Zhou R |Emerging β-sheet rich conformations in supercompact Huntingtin Exon-1 mutant structuresJ Am Chem Soc8820-8827139(26):20170705PMID:28609090DOI: 10.1021/jacs.7b00838Khakh BS, | Beaumont V, | Cachope R, | Munoz-Sanjuan I, | Goldman SA, | Grantyn R |Unravelling and exploiting astrocyte dysfunction in Huntington's diseaseTrends Neurosci422-43740(7):20170701PMID:28578789Dominguez D JF, | Poudel G, | Stout JC, | Gray M, | Chua P, | Borowsky B, | Egan GF, | Georgiou-Karistianis N |Longitudinal changes in the fronto-striatal network are associated with executive dysfunction and behavioral dysregulation in Huntington's disease : 30 months IMAGE-HD dataCortex139-14992():20170701PMID:28482181Nikan M, | Osborn MF, | Coles AH, | Biscans A, | Godinho BMDC, | Haraszti RA, | Sapp E, | Echeverria D, | DiFiglia M, | Aronin N, | Khvorova A |Synthesis and evaluation of parenchymal retention and efficacy of a metabolically stable O-Phosphocholine-N-docosahexaenoyl-l-serine siRNA conjugate in mouse brainBioconjug Chem1758-176628(6):20170621PMID:28462988Teka WW, | Hamade KC, | Barnett WH, | Kim T, | Markin SN, | Rybak IA, | Molkov YI |From the motor cortex to the movement and back againPLoS Onee017928812(6):20170620PMID:28632736PMCID: 5478113Byrne LM, | Rodrigues FB, | Blennow K, | Durr A, | Leavitt BR, | Roos RAC, | Scahill RI, | Tabrizi SJ, | Zetterberg H, | Langbehn D, | Wild EJ |Neurofilament light protein in blood as a potential biomarker of neurodegeneration in Huntington's disease: a retrospective cohort analysisLancet NeurolS1474-442217(30124-2):20170607PMID:28601473Quinti 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 |KEAP1-modifying small molecule reveals muted NRF2 signaling responses in neural stem cells from Huntington's disease patientsProc Natl Acad Sci USAE4676-E4685114(23):20170606PMID:28533375Cleary JD, | Ranum LP |New developments in RAN translation: insights from multiple diseasesCurr Opin Genet Dev125-13444():20170601PMID:28365506Fazio P, | Schain M, | Mrzljak L, | Amini N, | Nag S, | Al-Tawil N, | Fitzer-Attas CJ, | Bronzova J, | Landwehrmeyer B, | Sampaio C, | Halldin C, | Varrone A |Patterns of age related changes for phosphodiesterase type-10A in comparison with dopamine D(2/3) receptors and sub-cortical volumes in the human basal ganglia: A PET study with (18)F-MNI-659 and (11)C-raclopride with correction for partial volume effectNeuroimage330-339152():20170515PMID:28254508Neueder A, | Landles C, | Ghosh R, | Howland D, | Myers RH, | Faull RLM, | Tabrizi SJ, | Bates GP |The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington's disease patientsSci Rep13077(1):20170502PMID:28465506PMCID: 5431000Chiki A, | DeGuire SM, | Ruggeri FS, | Sanfelice D, | Ansaloni A, | Wang ZM, | Cendrowska U, | Burai R, | Vieweg S, | Pastore A, | Dietler G, | Lashuel HA |Mutant exon1 Huntingtin aggregation is regulated by T3 phosphorylation-induced structural changes and crosstalk between T3 phosphorylation and acetylation at K6Angew Chem Int Ed Engl5202-520756(19):20170502PMID:28334491HD iPSC ConsortiumDevelopmental alterations in Huntington's disease neural cells and pharmacological rescue in cells and miceNat Neurosci648-66020(5):20170501PMID:28319609DOI: 10.1038/nn.4532PMCID: 5610046Martín-Ibáñez R, | Guardia I, | Pardo M, | Herranz C, | Zietlow R, | Vinh NN, | Rosser A, | Canals JM |Insights in spatio-temporal characterization of human fetal neural stem cellsExp Neurol20-35291():20170501PMID:28131724Miller JRC, | Pfister EL, | Liu W, | Andre R, | Träger U, | Kennington LA, | Lo K, | Dijkstra S, | Macdonald D, | Ostroff G, | Aronin N, | Tabrizi SJ |Allele-selective suppression of mutant Huntingtin in primary human blood cellsSci Rep467407():20170424PMID:28436437DOI: 10.1038/srep46740PMCID: 5402279Zhang L, | Kang H, | Vázquez FX, | Toledo-Sherman L, | Luan B. | Zhou R |Molecular mechanism of stabilizing the helical structure of Huntingtin N17 in a micellar environmentJ Phys Chem B():20170417PMID:28413881Gregory S, | Long JD, | Klöppel S, | Razi A, | Scheller E, | Minkova L, | Papoutsi M, | Mills JA, | Durr A, | Leavitt BR, | Roos RAC, | Stout JC, | Scahill RI, | Langbehn DR, | Tabrizi SJ, | Rees G |Operationalizing compensation over time in neurodegenerative diseaseBrain1158-1165140(4):20170401PMID:28334888DOI: 10.1093/brain/awx022PMCID: 5382953Häggkvist J, | Tóth M, | Tari L, | Varnäs K, | Svedberg M, | Forsberg A, | Nag S, | Dominguez C, | Munoz-Sanjuan I, | Bard J, | Wityak J, | Varrone A, | Halldin C, | Mrzljak L 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