CHDI
2020
Authors
Title
Journal
- Comella-Bolla A, | Orlandi JG, | Miguez A, | Straccia M, | García-Bravo M, | Bombau G, | Galofré M, | Sanders P, | Carrere J, | Segovia JC, | Blasi J, | Allen ND, | Alberch J, | Soriano J, | Canals JM |Human pluripotent stem cell-derived neurons are functionally mature in vitro and integrate into the mouse striatum following transplantationMol Neurobiol2766-279857(6):20200602PMID:32356172Scahill RI, | Zeun P, | Osborne-Crowley K, | Johnson EB, | Gregory S, | Parker C, | Lowe J, | Nair A, | O'Callaghan C, | Langley C, | Papoutsi M, | McColgan P, | Estevez-Fraga C, | Fayer K, | Wellington H, | Rodrigues FB, | Byrne LM, | Heselgrave A, | Hyare H, | Sampaio C, | Zetterberg H, | Zhang H, | Wild EJ, | Rees G, | Robbins TW, | Sahakian BJ, | Langbehn D, | Tabrizi SJ |Biological and clinical characteristics of gene carriers far from predicted onset in the Huntington's disease Young Adult Study (HD-YAS): a cross-sectional analysisLancet Neurol502-51219(6):20200601PMID:32470422Wijeratne PA, | Johnson EB, | Eshaghi A, | Aksman L, | Gregory S, | Johnson HJ, | Poudel GR, | Mohan A, | Sampaio C, | Georgiou-Karistianis N, | Paulsen JS, | Tabrizi SJ, | Scahill RI, | IMAGE-HD, | PREDICT-HD and TRACK-HD investigators, | Alexander DC |Robust markers and sample sizes for multicenter trials of Huntington diseaseAnn Neurol751-76287(5):20200501PMID:32105364DOI: 10.1002/ana.25709Ellis N, | Tee A, | McAllister B, | Massey T, | McLauchlan D, | Stone T, | Correia K, | Loupe J, | Kim KH, | Barker D, | Hong EP, | Chao MJ, | Long JD, | Lucente D, | Vonsattel JPG, | Pinto RM, | Elneel KA, | Ramos EM, | Mysore JS, | Gillis T, | Wheeler VC, | Medway C, | Hall L, | Kwak S, | Sampaio C, | Ciosi M, | Maxwell A, | Chatzi A, | Monckton DG, | Orth M, | Landwehrmeyer GB, | Paulsen JS, | Shoulson I, | Myers RH, | van Duijn E, | Rickards H, | MacDonald ME, | Lee JM, | Gusella JF, | Jones L, | Holmans P |Genetic risk underlying psychiatric and cognitive symptoms in Huntington's diseaseBiol Psychiatry857-86587(9):20200501PMID:32087949Furlong LS, | Jakabek D, | Power BD, | Owens-Walton C, | Wilkes FA, | Walterfang M, | Velakoulis D, | Egan G, | Looi JC, | Georgiou-Karistianis N |Morphometric in vivo evidence of thalamic atrophy correlated with cognitive and motor dysfunction in Huntington's disease: The IMAGE-HD studPsychiatry Res Neuroimaging111048298():20200430PMID:32120305Nath SR, | Lieberman ML, | Yu Z, | Marchioretti C, | Jones ST, | Danby ECE, | Van Pelt KM, | Sorarù G, | Robins DM, | Bates GP, | Pennuto M, | Lieberman AP |MEF2 impairment underlies skeletal muscle atrophy in polyglutamine diseaseActa Neuropathol1-18():20200417PMID:32306066PMCID: 7166004Di Marco A, | Vignone D, | Gonzalez Paz O, | Fini I, | Battista MR, | Cellucci A, | Bracacel E, | Auciello G, | Veneziano M, | Khetarpal V, | Rose M, | Rosa A, | Gloaguen I, | Monteagudo E, | Herbst T, | Dominguez C, | Muñoz-Sanjuán I |Establishment of an in vitro human blood-brain barrier model derived from induced pluripotent stem cells and comparison to a porcine cell-based systemCellsE9949(4):20200416PMID:32316221DOI: 10.3390/cells9040994Smith-Geater C, | Hernandez SJ, | Lim RG, | Adam M, | Wu J, | Stocksdale JT, | Wassie BT, | Gold MP, | Wang KQ, | Miramontes R, | Kopan L, | Orellana I, | Joy S, | Kemp PJ, | Allen ND, | Fraenkel E, | Thompson LM |Aberrant development corrected in adult-onset Huntington's disease iPSC-derived neuronal cultures via WNT signaling modulationStem Cell Reports406-41914(3):20200310PMID:32109367PMCID: 7066322Morton AJ, | Middleton B, | Rudiger S, | Bawden CS, | Kuchel TR, | Skene DJ |Increased plasma melatonin in presymptomatic Huntington disease sheep (Ovis aries): Compensatory neuroprotection in a neurodegenerative disease?J Pineal Rese1262468(2):20200302PMID:31742766DOI: 10.1111/jpi.12624Mégret L, | Nair SS, | Dancourt J, | Aaronson J, | Rosinski J, | Neri C |Combining feature selection and shape analysis uncovers precise rules for miRNA regulation in Huntington's disease miceBMC Bioinformatics7521(1):20200224PMID:32093602Ghavami A, | Olsen M, | Kwan M, | Beltran J, | Shea J, | Ramboz S, | Duan W, | Lavery D, | Howland D, | Park LC |Transcriptional assessment of striatal mRNAs as valid biomarkers of disease progression in three mouse models of Huntington's diseaseJ Huntingtons Dis13-319(1):20200210PMID:32007959DOI: 10.3233/JHD-190389Alexander B, | Georgiou-Karistianis N, | Beare R, | Ahveninen LM, | Lorenzetti V, | Stout JC, | Glikmann-Johnston Y |Accuracy of automated amygdala MRI segmentation approaches in Huntington's disease in the IMAGE-HD cohortHum Brain Mapp():20200207PMID:32034838DOI: 10.1002/hbm.24918Messer A, | Butler DC |Optimizing intracellular antibodies (intrabodies/nanobodies) to treat neurodegenerative disordersNeurobiol Dis104619134():20200203PMID:31669671McAdam RL, | Morton A, | Gordon SL, | Alterman JF, | Khvorova A, | Cousin MA, | Smillie KJ |Loss of huntingtin function slows synaptic vesicle endocytosis in striatal neurons from the htt (Q140/Q140) mouse model of Huntington's diseaseNeurobiol Dis104637134():20200203PMID:31614197Batista AR, | King OD, | Reardon CP, | Davis C, | Shankaracharya, | Philip V, | Gray-Edwards H, | Aronin N, | Lutz C, | Landers J, | Sena-Esteves M |Ly6a differential expression in blood-brain barrier is responsible for strain specific central nervous system transduction profile of AAV-PHP.B.Hum Gene Ther90-10231(1-2):20200130PMID:31696742DOI: 10.1089/hum.2019.186Polosecki P, | Castro E, | Rish I, | Pustina D, | Warner JH, | Wood A, | Sampaio C, | Cecchi GA |Resting-state connectivity stratifies premanifest Huntington's disease by longitudinal cognitive decline rateSci Rep125210(1):20200127PMID:31988371Fazio P, | Fitzer-Attas CJ, | Mrzljak L, | Bronzova J, | Nag S, | Warner JH, | Landwehrmeyer B, | Al-Tawil N, | Halldin C, | Forsberg A, | Ware J, | Dilda V, | Wood A, | Sampaio C, | Varrone A, | PEARL-HD and LONGPDE10 collaborators |PET molecular imaging of phosphodiesterase 10A: An early biomarker of Huntington's disease progressionMov Disord606-61535(4):20200122PMID:31967355DOI: 10.1002/mds.27963Kedaigle AJ, | Reidling JC, | Lim RG, | Adam M, | Wu J, | Wassie B, | Stocksdale JT, | Casale MS, | Fraenkel E, | Thompson LM |Treatment with JQ1, a BET bromodomain inhibitor, is selectively detrimental to R6/2 Huntington's disease miceHum Mol Genet202-21529(2):20200120PMID:31696228DOI: 10.1093/hmg/ddz264Chongtham A, | Bornemann DJ, | Barbaro BA, | Lukacsovich T, | Agrawal N, | Syed A, | Worthge S, | Purcell J, | Burke J, | Chin TM, | Marsh JL |Effects of flanking sequences and cellular context on subcellular behavior and pathology of mutant HTTHum Mol Genet():20200116PMID:31943010DOI: 10.1093/hmg/ddaa001Julayanont P, | McFarland NR, | Heilman KM |Mild cognitive impairment and dementia in motor manifest Huntington's disease: Classification and prevalenceJ Neurol Sci116523408():20200115PMID:31678902Cariulo C, | Verani M, | Martufi P, | Ingenito R, | Finotto M, | Deguire SM, | Lavery DJ, | Toledo-Sherman L, | Lee R, | Doherty EM, | Vogt TF, | Dominguez C, | Lashuel HA, | Petricca L, | Caricasole A |Ultrasensitive quantitative measurement of huntingtin phosphorylation at residue S13Biochem Biophys Res Commun549-554521(3):20200115PMID:31677786Bertoglio D, | Verhaeghe J, | Korat Š, | Miranda A, | Cybulska K, | Wyffels L, | Stroobants S, | Mrzljak L, | Dominguez C, | Skinbjerg M, | Liu L, | Munoz-Sanjuan I, | Staelens S |Elevated type 1 metabotropic glutamate receptor availability in a mouse model of Huntington's disease: a longitudinal PET studyMol Neurobiol():20200110PMID:31912442Julayanont P, | Heilman KM, | McFarland NR|Early-motor phenotype relates to neuropsychiatric and cognitive disorders in Huntington's diseaseMov Disord():20200110PMID:31922295DOI: 10.1002/mds.27980Pflanz CP, | Charquero-Ballester M, | Majid DSA, | Winkler AM, | Vallée E, | Aron AR, | Jenkinson M, | Douaud G |One-year changes in brain microstructure differentiate preclinical Huntington's disease stagesNeuroimage Clin10209925():20200106PMID:31865023
2019
AuthorsTitleJournal- Aviolat H, | Pinto RM, | Godschall E, | Murtha R, | Richey HE, | Sapp E, | Vodicka P, | Wheeler VC, | Kegel-Gleason KB, | DiFiglia M |Assessing average somatic CAG repeat instability at the protein levelSci Rep191529(1):20191216PMID:31844074Chandra A, | Valkimadi PE, | Pagano G, | Cousins O, | Dervenoulas G, | Politis M, | Alzheimer's Disease Neuroimaging Initiative |Applications of amyloid, tau, and neuroinflammation PET imaging to Alzheimer's disease and mild cognitive impairmentHum Brain Mapp5424-544240(18):20191215PMID:31520513DOI: 10.1002/hbm.24782Ardan T, | Baxa M, | Levinská B, | Sedláčková M, | Nguyen TD, | Klíma J, | Juhás Š, | Juhásová J, | Šmatlíková P, | Vochozková P, | Motlík J, | Ellederová Z |Transgenic minipig model of Huntington's disease exhibiting gradually progressing neurodegenerationDis Model Mechdmm04131913(2):20191212PMID:31645369DOI: 10.1242/dmm.041319PMCID: 6918760Bertoglio D, | Verhaeghe J, | Korat Š, | Miranda A, | Wyffels L, | Stroobants S, | Mrzljak L, | Dominguez C, | Liu L, | Skinbjerg M, | Munoz-Sanjuan I, | Staelens S |In vitro and In vivo assessment of suitable reference region and kinetic modelling for the mGluR1 radioligand [(11)C] ITDM in miceMol Imaging Biol():20191202PMID:31792838Estrada-Sánchez AM, | Blake CL, | Barton SJ, | Howe AG, | Rebec GV |Lack of mutant huntingtin in cortical efferents improves behavioral inflexibility and corticostriatal dynamics in Huntington's disease miceJ Neurophysiol2621-2629122(6):20191202PMID:31693428PMCID: 6966319Smarr B, | Cutler T, | Loh DH, | Kudo T, | Kuljis D, | Kriegsfeld L, | Ghiani CA, | Colwell CS |Circadian dysfunction in the Q175 model of Huntington's disease: Network analysisJ Neurosci Res1606-162397(12):20191202PMID:31359503DOI: 10.1002/jnr.24505Papadopoulou AS, | Gomez-Paredes C, | Mason MA, | Taxy BA, | Howland D, | Bates GP |Extensive expression analysis of Htt transcripts in brain regions from the zQ175 HD mouse model using a QuantiGene multiplex assaySci Rep161379(1):20191106PMID:31695145Irvine EE, | Katsouri L, | Plattner F, | Al-Qassab H, | Al-Nackkash R, | Bates GP, | Withers DJ |Genetic deletion of S6k1 does not rescue the phenotypic deficits observed in the R6/2 mouse model of Huntington's diseaseSci Rep161339(1):20191106PMID:31695068PMCID: 6834565Marottoli FM, | Priego M, | Flores-Barrera E, | Pisharody R, | Zaldua S, | Fan KD, | Ekkurthi GK, | Brady ST, | Morfini GA, | Tseng KY, | Tai LM |EGF treatment improves motor behavior and cortical GABAergic function in the R6/2 mouse model of Huntington's diseaseMol Neurobiol7708-771856(11):20191105PMID:31104296PMCID: 6815691Gregory S, | Odish OFF, | Mayer I, | Mills J, | Johnson EB, | Scahill RI, | Rothwell J, | Rees G, | Long JD, | Tabrizi SJ, | Roos RAC, | Orth M |Multimodal characterization of the visual network in Huntington's disease gene carriersClin Neurophysiol2053-2059130(11):20191101PMID:31541982Ciosi M, | Maxwell A, | Cumming SA, | Hensman Moss DJ, | Alshammari AM, | Flower MD, | Durr A, | Leavitt BR, | Roos RAC, | TRACK-HD team, | Enroll-HD team | Holmans P, | Jones L, | Langbehn DR, | Kwak S, | Tabrizi SJ, | Monckton DG |A genetic association study of glutamine-encoding DNA sequence structures, somatic CAG expansion, and DNA repair gene variants, with Huntington disease clinical outcomesEBioMedicine568-58048():20191030PMID:31607598Diaz-Castro B, | Gangwani MR, | Yu X, | Coppola G, | Khakh BS |Astrocyte molecular signatures in Huntington's diseaseSci Transl Medeaaw854611(514):20191016PMID:31619545Haremaki T, | Metzger JJ, | Rito T, | Ozair MZ, | Etoc F, | Brivanlou AH |Self-organizing neuruloids model developmental aspects of Huntington's disease in the ectodermal compartmentNat Biotechnol1198-120837(10):20191011PMID:31501559Kim KH, | Abu Elneel K, | Shin JW, | Keum JW, | Seong D, | Kwak S, | Lee R, | Gusella JF, | MacDonald ME, | Seong IS, | Lee JM |Full sequence of mutant huntingtin 3'-untranslated region and modulation of its gene regulatory activity by endogenous microRNAJ Hum Genet995-100464(10):20191001PMID:31296921Sap KA, | Guler AT, | Bezstarosti K, | Bury AE, | Juenemann K, | Demmers JAA, | Reits EA |Global proteome and ubiquitinome changes in the soluble and insoluble fractions of Q175 Huntington mice brainsMol Cell Proteomics1705-172018(9):20190930PMID:31138642PMCID: 6731087Khakh BS |Astrocyte-neuron interactions in the striatum: insights on identity, form, and functionTrends Neurosci617-63042(9):20190904PMID:31351745van der Plas E, | Langbehn DR, | Conrad AL, | Koscik TR, | Tereshchenko A, | Epping EA, | Magnotta VA, | Nopoulos PC |Abnormal brain development in child and adolescent carriers of mutant huntingtinNeurologye1021-e103093(10):20190903PMID:31371571Kubera KM, | Schmitgen MM, | Hirjak D, | Wolf RC, | Orth M |Cortical neurodevelopment in pre-manifest Huntington's diseaseNeuroimage Clin10191323():20190902PMID:31491822PMCID: 6627026Wanker EE, | Ast A, | Schindler F, | Trepte P, | Schnoegl S |The pathobiology of perturbed mutant huntingtin protein-protein interactions in Huntington's diseaseJ Neurochem():20190816PMID:31418858DOI: 10.1111/jnc.14853Swainson LA, | Ahn H, | Pajanirassa P, | Khetarpal V, | Deleage C, | Estes JD, | Hunt PW, | Munoz-Sanjuan I, | McCune JM |Kynurenine 3-Monooxygenase inhibition during acute simian immunodeficiency virus infection lowers PD-1 expression and improves post-combination antiretroviral therapy CD4(+) T cell counts and body weightJ Immunol899-910203(4):20190815PMID:31285277PMCID: 6684450Langbehn DR, | Stout JC, | Gregory S, | Mills JA, | Durr A, | Leavitt BR, | Roos RAC, | Long JD, | Owen G, | Johnson HJ, | Borowsky B, | Craufurd D, | Reilmann R, | Landwehrmeyer GB, | Scahill RI, | Tabrizi SJ, | TRACK-HD and Track-On HD Groups |Association of CAG repeats with long-term progression in Huntington diseaseJAMA Neurol():20190812PMID:31403680Federspiel JD, | Greco TM, | Lum KK, | Cristea IM |Hdac4 interactions in Huntington's disease viewed through the prism of MultiomicsMol Cell ProteomicsS92-S11318(8):20190809PMID:31040226GeM-HD Consortium, | Lee JM, | Correia K, | Loupe J, | Kim KH, | Barker D, | Hong EP, | Chao MJ, | Long JD, | Lucente D, | Vonsattel JPG, | Pinto RM, | Abu Elneel K, | Ramos EM, | Mysore JS, | Gillis T, | Wheeler VC, | MacDonald ME, | Gusella JF, | McAllister B, | Massey T, | Medway C, | Stone TC, | Hall L, | Jones L, | Holmans P, | Kwak S, | Ehrhardt AG, | Sampaio C, | Ciosi M, | Maxwell A, | Chatzi A, | Monckton DG, | Orth M, | Landwehrmeyer GB, | Paulsen JS, | Dorsey ER, | Shoulson I, | Myers RH |CAG repeat not polyglutamine length determines timing of Huntington's disease onsetCell887-900178(4):20190808PMID:31398342PMCID: 6700281Huhtala T, | Poutiainen P, | Rytkönen J, | Lehtimäki K, | Parkkari T, | Kasanen I, | Airaksinen AJ, | Koivula T, | Sweeney P, | Kontkanen O, | Wityak J, | Dominiquez C, | Park LC |Improved synthesis of [(18)F] fallypride and characterization of a Huntington's disease mouse model, zQ175DN KI, 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