CHDI
2019
Authors
Title
Journal
- Padovan-Neto FE, | Jurkowski L, | Murray C, | Stutzmann GE, | Kwan M, | Ghavami A, | Beaumont V, | Park LC, | West AR |Age- and sex-related changes in cortical and striatal nitric oxide synthase in the Q175 mouse model of Huntington's diseaseNitric Oxide40-5083():20190201PMID:30528913Espinoza FA, | Liu J, | Ciarochi J, | Turner JA, | Vergara VM, | Caprihan A, | Misiura M, | Johnson HJ, | Long JD, | Bockholt JH, | Paulsen JS, | Calhoun VD |Dynamic functional network connectivity in Huntington's disease and its associations with motor and cognitive measuresHum Brain Mapp1-14():20190107PMID:30618191DOI: 10.1002/hbm.24504Lu M, | Banetta L, | Young LJ, | Smith EJ, | Bates GP, | Zaccone A, | Kaminski Schierle GS, | Tunnacliffe A, | Kaminski CF |Live-cell super-resolution microscopy reveals a primary role for diffusion in polyglutamine-driven aggresome assemblyJ Biol Chem257-268294(1):20190104PMID:30401748PMCID: 6322900Osipovitch M, | Asenjo Martinez A, | Mariani JN, | Cornwell A, | Dhaliwal S, | Zou L, | Chandler-Militello D, | Wang S, | Li X, | Benraiss SJ, | Agate R, | Lampp A, | Benraiss A, | Windrem MS, | Goldman SA |Human ESC-derived chimeric mouse models of Huntington's disease reveal cell-intrinsic defects in glial progenitor cell differentiationCell Stem Cell107-12224(1):20190103PMID:30554964Luckhurst CA, | Aziz O, | Beaumont V, | Bürli RW, | Breccia P, | Maillard MC, | Haughan AF, | Lamers M, | Leonard P, | Matthews KL, | Raphy G, | Stott AJ, | Munoz-Sanjuan I, | Thomas B, | Wall M, | Wishart G, | Yates D, | Dominguez C |Development and characterization of a CNS-penetrant benzhydryl hydroxamic acid class IIa histone deacetylase inhibitorBioorg Med Chem Lett83-8829(1):20190101PMID:30463802
2018
AuthorsTitleJournal- Wang D, | Zhong L, | Li M, | Li J, | Tran K, | Ren L, | He R, | Xie J, | Moser RP, | Fraser C, | Kuchel T, | Sena-Esteves M, | Flotte TR, | Aronin N, | Gao G |Adeno-associated virus neutralizing antibodies in large animals and their impact on brain intraparenchymal gene transferMol Ther Methods Clin Dev65-7211():20181214PMID:30397628PMCID: 6205343McBride SD, | Morton AJ |Indices of comparative cognition: assessing animal models of human brain functionExp Brain Res3379-3390236(12):20181203PMID:30267138PMCID: 6267686Kuljis D, | Kudo T, | Tahara Y, | Ghiani CA, | Colwell CS |Pathophysiology in the suprachiasmatic nucleus in mouse models of Huntington's diseaseJ Neurosci Res1862-187596(12):20181203PMID:30168855DOI: 10.1002/jnr.24320Mondo E, | Moser R, | Gao G, | Mueller C, | Sena-Esteves M, | Sapp E, | Pfister E, | O'Connell D, | Takle K, | Erger KE, | Liu W, | Conlon TJ, | DiFiglia M, | Gounis MJ, | Aronin N |Selective neuronal uptake and distribution of AAVrh8, AAV9, and AAVrh10 in sheep after intra-striatal administrationJ Huntingtons Dis309-3197(4):20181124PMID:30320596DOI: 10.3233/JHD-180302Long JD, | Mills JA |Joint modeling of multivariate longitudinal data and survival data in several observational studies of Huntington's diseaseBMC Med Res Methodol13818(1):20181116PMID:30445915PMCID: 6240282Godinho BMDC, | Henninger N, | Bouley J, | Alterman JF, | Haraszti RA, | Gilbert JW, | Sapp E, | Coles AH, | Biscans A, | Nikan M, | Echeverria D, | DiFiglia M, | Aronin N, | Khvorova A |Transvascular delivery of hydrophobically modified siRNAs: gene silencing in the rat brain upon disruption of the blood-brain barrierMol Ther2580-259126(11):20181107PMID:30143435Rocha NP, | Mwangi B, | Gutierrez Candano CA, | Sampaio C, | Furr Stimming E, | Teixeira AL |The clinical picture of psychosis in manifest Huntington's disease: a comprehensive analysis of the Enroll-HD databaseFront Neurol9(930):20181106PMID:30459704PMCID: 6232301Zhang J, | Gregory S, | Scahill RI, | Durr A, | Thomas DL, | Lehericy S, | Rees G, | Tabrizi SJ, | Zhang H, | TrackOn-HD investigators |In vivo characterization of white matter pathology in premanifest huntington's diseaseAnn Neurol497-50484(4):20181029PMID:30063250DOI: 10.1002/ana.25309PMCID: 6221120Piiponniemi TO, | Parkkari T, | Heikkinen T, | Puoliväli J, | Park LC, | Cachope R, | Kopanitsa MV |Impaired performance of the Q175 mouse model of Huntington's disease in the touch screen paired associates learning taskFront Behav Neurosci12(226):20181002PMID:30333735PMCID: 6176131Aron R, | Pellegrini P, | Green EW, | Maddison DC, | Opoku-Nsiah K, | Wong JS, | Daub AC, | Giorgini F, | Finkbeiner S |Deubiquitinase Usp12 functions noncatalytically to induce autophagy and confer neuroprotection in models of Huntington's diseaseNat Commun31919(1):20180928PMID:30266909PMCID: 6162324Neueder A, | Dumas AA, | Benjamin AC, | Bates GP |Regulatory mechanisms of incomplete huntingtin mRNA splicingNat Commun39559(1):20180927PMID:30262848PMCID: 6160442Byrne LM, | Rodrigues FB, | Johnson EB, | Wijeratne PA, | De Vita E, | Alexander DC, | Palermo G, | Czech C, | Schobel S, | Scahill RI, | Heslegrave A, | Zetterberg H, | Wild EJ |Evaluation of mutant huntingtin and neurofilament proteins as potential markers in Huntington's diseaseSci Transl Medeaat710810(458):20180912PMID:30209243Ast 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 |mHTT seeding activity: a marker of disease progression and neurotoxicity in models of Huntington's diseaseMol Cell675-68871(5):20180906PMID:30193095Evers MM, | Miniarikova J, | Juhas S, | Vallès A, | Bohuslavova B, | Juhasova J, | Skalnikova HK, | Vodicka P, | Valekova I, | Brouwers C, | Blits B, | Lubelski J, | Kovarova H, | Ellederova Z, | van Deventer SJ, | Petry H, | Motlik J, | Konstantinova P |AAV5-miHTT gene therapy demonstrates broad distribution and strong human mutant huntingtin lowering in a Huntington's disease minipig modelMol Ther2163-217726(9):20180905PMID:30007561PMCID: 6127509Didiot MC, | Ferguson CM, | Ly S, | Coles AH, | Smith AO, | Bicknell AA, | Hall LM, | Sapp E, | Echeverria D, | Pai AA, | DiFiglia M, | Moore MJ, | Hayward LJ, | Aronin N, | Khvorova A |Nuclear localization of Huntingtin mRNA is specific to cells of neuronal originCell Rep2553-256024(10):20180904PMID:30184490Goodliffe JW, | Song H, | Rubakovic A, | Chang W, | Medalla M, | Weaver CM, | Luebke JI |Differential changes to D1 and D2 medium spiny neurons in the 12-month-old Q175+/- mouse model of Huntington's diseasePLoS Onee020062613(8):20180817PMID:30118496PMCID: 6097649Haraszti RA, | Miller R, | Didiot MC, | Biscans A, | Alterman JF, | Hassler MR, | Roux L, | Echeverria D, | Sapp E, | DiFiglia M, | Aronin N, | Khvorova A |Optimized cholesterol-siRNA chemistry improves productive loading onto extracellular vesiclesMol Ther1973-198226(8):20180801PMID:29937418Pido-Lopez J, | Andre R, | Benjamin AC, | Ali N, | Farag S, | Tabrizi SJ, | Bates GP |In vivo neutralization of the protagonist role of macrophages during the chronic inflammatory stage of Huntington's diseaseSci Rep114478(1):20180730PMID:30061661Mir A, | Alterman JF, | Hassler MR, | Debacker AJ, | Hudgens E, | Echeverria D, | Brodsky MH, | Khvorova A, | Watts JK, | Sontheimer EJ |Heavily and fully modified RNAs guide efficient SpyCas9-mediated genome editingNat Commun26419(1):20180706PMID:29980686Ko J, | Isas JM, | Sabbaugh A, | Yoo JH, | Pandey NK, | Chongtham A, | Ladinsky M, | Wu WL, | Rohweder H, | Weiss A, | Macdonald D, | Munoz-Sanjuan I, | Langen R, | Patterson PH, | Khoshnan A |Identification of distinct conformations associated with monomers and fibril assemblies of mutant huntingtinHum Mol Genet2330–234327(13):20180701PMID:29912367DOI: 10.1093/hmg/ddy141Verhaeghe J, | Bertoglio D, | Kosten L, | Thomae D, | Verhoye M, | Van Der Linden A, | Wyffels L, | Stroobants S, | Wityak J, | Dominguez C, | Mrzljak L, | Staelens S |Noninvasive relative quantification of [(11)C]ABP688 PET imaging in mice versus an input function measured over an arteriovenous shuntFront Neurol9(516):20180629PMID:30013509PMCID: 6036254Reif A, | Chiki A, | Ricci J, | Lashuel HA |Generation of native, untagged Huntingtin Exon1 monomer and fibrils using a SUMO fusion strategyJ Vis Exp136():20180627PMID:30010666DOI: 10.3791/57506Biscans A, | Haraszti RA, | Echeverria D, | Miller R, | Didiot MC, | Nikan M, | Roux L, | Aronin N, | Khvorova A |Hydrophobicity of lipid-conjugated siRNAs predicts productive loading to small extracellular vesiclesMol Ther1520-152826(6):20180606PMID:29699940Shin B, | Jung R, | Oh H, | Owens GE, | Lee H, | Kwak S, | Lee R, | Cotman SL, | Lee JM, | MacDonald ME, | Song JJ, | Vijayvargia R, | Seong IS |Novel DNA aptamers that bind to mutant Huntingtin and modify its activityMol Ther Nucleic Acids416-42811():20180601PMID:29858077PMCID: 5992459Covey DP, | Dantrassy HM, | Yohn SE, | Castro A, | Conn PJ, | Mateo Y, | Cheer JF |Inhibition of endocannabinoid degradation rectifies motivational and dopaminergic deficits in the Q175 mouse model of Huntington's diseaseNeuropsychopharmacology2056–206343():20180601PMID:29925886PMCID: 6098121Suelves N, | Miguez A, | López-Benito S, | Barriga GG, | Giralt A, | Alvarez-Periel E, | Arévalo JC, | Alberch J, | Ginés S, | Brito V |Early downregulation of p75(NTR) by genetic and pharmacological approaches delays the onset of motor deficits and striatal dysfunction in Huntington's disease miceMol Neurobiol():20180528PMID:29804232Martin DDO, | Kay C, | Collins JA, | Nguyen YT, | Slama RA, | Hayden MR |A human huntingtin SNP alters post-translational modification and pathogenic proteolysis of the protein causing Huntington diseaseSci Rep80969(1):20180525PMID:29802276Bertoglio D, | Kosten L, | Verhaeghe J, | Thomae D, | Wyffels L, | Stroobants S, | Wityak J, | Dominguez C, | Mrzljak L, | Staelens S |Longitudinal characterization of mGluR5 using (11)C-ABP688 PET imaging in the Q175 mouse model of Huntington's diseaseJ Nucl Med1723-172759(11):20180524PMID:29794227McClory H, | Wang X, | Sapp E, | Gatune LW, | Iuliano M, | Wu CY, | Nathwani G, | Kegel-Gleason KB, | DiFiglia M, | Li X |The COOH-terminal domain of huntingtin interacts with RhoGEF kalirin and modulates cell survivalSci Rep8(1):20180522PMID:29789657Schuldenzucker V, | Schubert R, | Muratori LM, | Freisfeld F, | Rieke L, | Matheis T, | Schramke S, | Motlik J, | Kemper N, | Radespiel U, | Reilmann R |Behavioral assessment of stress compensation in minipigs transgenic for the Huntington gene using cortisol levels: a proof-of-concept studyJ Huntingtons Dis151-1617(2):20180519PMID:29843247DOI: 10.3233/JHD-180285Gregory S, | Long JD, | Klöppel S, | Razi A, | Scheller E, | Minkova L, | Johnson EB, | Durr A, | Roos RAC, | Leavitt BR, | Mills JA, | Stout JC, | Scahill RI, | Tabrizi SJ, | Rees G, | Track-On investigators |Testing a longitudinal compensation model in premanifest Huntington's diseaseBrain():20180518PMID:29788038DOI: 10.1093/brain/awy122Chao MJ, | Kim KH, | Shin JW, | Lucente D, | Wheeler VC, | Li H, | Roach JC, | Hood L, | Wexler NS, | Jardim LB, | Holmans P, | Jones L, | Orth M, | Kwak S, | MacDonald ME, | Gusella JF, | Lee JM |Population-specific genetic modification of Huntington's disease in VenezuelaPLoS Genete100727414(5):20180511PMID:29750799Mestre TA, | Busse M, | Davis AM, | Quinn L, | Rodrigues FB, | Burgunder JM, | Carlozzi NE, | Walker F, | Ho AK, | Sampaio C, | Goetz CG, | Cubo E, | Martinez-Martin P, | Stebbins GT, | Members of the MDS Committee on Rating Scales Development |Rating scales and performance-based measures for assessment of functional ability in Huntington's disease: critique and recommendationsMov Disord Clin Pract361-3725(4):20180509PMID:30363510DOI: 10.1002/mdc3.12617PMCID: 6174516Castro E, | Polosecki P, | Rish I, | Pustina D, | Warner JH, | Wood A, | Sampaio C, | Cecchi GA |Baseline multimodal information predicts future motor impairment in premanifest Huntington's diseaseNeuroimage Clin443-45319():20180509PMID:29984153PMCID: 6029560Gregory S, | Crawford H, | Seunarine K, | Leavitt B, | Durr A, | Roos RAC, | Scahill RI, | Tabrizi SJ, | Rees G, | Langbehn D, | Orth M |Natural biological variation of white matter microstructure is accentuated in Huntington's diseaseHum Brain Mapp():20180422PMID:29682858DOI: 10.1002/hbm.24191Beckmann 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:29660633Reiner A, | Deng YP |Disrupted striatal neuron inputs and outputs in Huntington's diseaseCNS Neurosci Ther250-28024(4):20180402PMID:29582587DOI: 10.1111/cns.12844PMCID: 5875736Wijeratne PA, | Young AL, | Oxtoby NP, | Marinescu RV, | Firth NC, | Johnson EB, | Mohan A, | Sampaio C, | Scahill RI, | Tabrizi SJ, | Alexander DC |An image-based model of brain volume biomarker changes in Huntington's diseaseAnn Clin Transl Neurol570-5825(5):20180402PMID:29761120DOI: 10.1002/acn3.558PMCID: 5945962Lai 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: 5868199Mestre TA, | Carlozzi NE, | Ho AK, | Burgunder JM, | Walker F, | Davis AM, | Busse M, | Quinn L, | Rodrigues FB, | Sampaio C, | Goetz CG, | Cubo E, | Martinez-Martin P, | Stebbins GT, | Members of the MDS Committee on Rating Scales Development|Quality of life in Huntington's disease: Critique and recommendations for measures assessing patient health-related quality of life and caregiver quality of lifeMov Disord742-74933(5):20180323PMID:29570848DOI: 10.1002/mds.27317Bresciani 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
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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: 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