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Center for Neurodegenerative Disease Research

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Microglia-mediated recovery from ALS-relevant motor neuron degeneration in a mouse model of TDP-43 proteinopathy

February 2018 | Krista J. Spiller, Clark R. Restrepo, Tahiyana Khan, Myrna A. Dominique, Terry C. Fang, Rebecca G. Canter, Christopher J. Roberts, Kelly R. Miller, Richard M. Ransohoff, John Q. Trojanowski, Virginia M.-Y. Lee



ABSTRACT: Though motor neurons selectively degenerate in Amyotrophic lateral sclerosis (ALS), other cell types are likely involved in this disease. We recently generated rNLS8 mice in which human TDP-43 (hTDP-43) pathology could be reversibly induced in neurons and expected that microglia would contribute to neurodegeneration. However, only subtle microglial changes were detected during disease in the spinal chord, despite progressive motor neuron loss; microglia still reacted to inflammatory triggers in these mice. Notably, after hTDP-43 expression was suppressed, microglia dramatically proliferated and changed their morphology and gene expression profiles. These abundant, reactive microglia selectively cleared neuronal hTDP-43. Finally, when microgliosis was blocked during the early recovery phase using PLX3397, a CSF1R and c-kit inhibitor, rNLS8 mice failed to regain full motor function, revealing an important neuroprotective role for microglia. Therefore, reactive microglia exert neuroprotective functions in this amyotrophic lateral sclerosis model, and definition of the underlying mechanism could point toward novel therapeutic strategies. 

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More CNDR & Neurodegenerative Disease-related Publications

Elevated CSF GAP-43 is Alzheimer's disease specific and associated with tau and amyloid pathology.

Alzheimers Dement. 2018 Oct 12; Authors: Sandelius Å, Portelius E, Källén Å, Zetterberg H, Rot U, Olsson B, Toledo JB, Shaw LM, Lee VMY, Irwin DJ, Grossman M, Weintraub D, Chen-Plotkin A, Wolk DA, McCluskey L, Elman L, Kostanjevecki V, Vandijck M, McBride J, Trojanowski JQ, Blennow K


Patient-derived frontotemporal lobar degeneration brain extracts induce formation and spreading of TDP-43 pathology in vivo.

Nat Commun. 2018 Oct 11; Authors: Porta S, Xu Y, Restrepo CR, Kwong LK, Zhang B, Brown HJ, Lee EB, Trojanowski JQ, Lee VM


Elevated YKL-40 and low sAPPβ:YKL-40 ratio in antemortem cerebrospinal fluid of patients with pathologically confirmed FTLD.

J Neurol Neurosurg Psychiatry. 2018 Oct 08; Authors: Alcolea D, Irwin DJ, Illán-Gala I, Muñoz L, Clarimón J, McMillan CT, Fortea J, Blesa R, Lee EB, Trojanowski JQ, Grossman M, Lleó A


Focal ultrasound strikes a new tune for targeted drug delivery.

Mov Disord. 2018 Oct 04; Authors: Luk KC


UNC13A polymorphism contributes to frontotemporal disease in sporadic amyotrophic lateral sclerosis.

Neurobiol Aging. 2018 Sep 27; Authors: Placek K, Baer GM, Elman L, McCluskey L, Hennessy L, Ferraro PM, Lee EB, Lee VMY, Trojanowski JQ, Van Deerlin VM, Grossman M, Irwin DJ, McMillan CT


Prevalence of amyloid-β pathology in distinct variants of primary progressive aphasia.

Ann Neurol. 2018 Sep 26; Authors: Bergeron D, Gorno-Tempini ML, Rabinovici GD, Santos-Santos MA, Seeley W, Miller BL, Pijnenburg Y, Keulen MA, Groot C, van Berckel BNM, van der Flier WM, Scheltens P, Rohrer JD, Warren JD, Schott JM, Fox NC, Sanchez-Valle R, Grau-Rivera O, Gelpi E, Seelaar H, Papma JM, van Swieten JC, Hodges JR, Leyton CE, Piguet O, Rogalsky EJ, Mesulam MM, Koric L, Nora K, Pariente J, Dickerson B, Mackenzie IR, Hsiung GR, Belliard S, Irwin DJ, Wolk DA, Grossman M, Jones M, Harris J, Mann D, Snowden JS, Chrem-Mendez P, Calandri IL, Amengual AA, Miguet-Alfonsi C, Magnin E, Magnani G, Santangelo R, Deramecourt V, Pasquier F, Mattsson N, Nilsson C, Hansson O, Keith J, Masellis M, Black SE, Matías-Guiu JA, Cabrera-Martin MN, Paquet C, Dumuirger J, Teichmann M, Sarazin M, Bottlaender M, Dubois B, Rowe CC, Villemagne VL, Vandenberghe R, Granadillo E, Teng E, Mendez M, Meyer PT, Frings L, Lleó A, Blesa R, Fortea J, Seo SW, Diehl-Schmid J, Grimmer T, Frederiksen KS, Sánchez-Juan P, Chételat G, Jansen W, Bouchard RW, Robert L, Visser PJ, Ossenkoppele R


Non-beta-amyloid/tau cerebrospinal fluid markers inform staging and progression in Alzheimer's disease.

Alzheimers Res Ther. 2018 Sep 25; Authors: Gangishetti U, Christina Howell J, Perrin RJ, Louneva N, Watts KD, Kollhoff A, Grossman M, Wolk DA, Shaw LM, Morris JC, Trojanowski JQ, Fagan AM, Arnold SE, Hu WT


A C6orf10/LOC101929163 locus is associated with age of onset in C9orf72 carriers.

Brain. 2018 Sep 25; Authors: Zhang M, Ferrari R, Tartaglia MC, Keith J, Surace EI, Wolf U, Sato C, Grinberg M, Liang Y, Xi Z, Dupont K, McGoldrick P, Weichert A, McKeever PM, Schneider R, McCorkindale MD, Manzoni C, Rademakers R, Graff-Radford NR, Dickson DW, Parisi JE, Boeve BF, Petersen RC, Miller BL, Seeley WW, van Swieten JC, van Rooij J, Pijnenburg Y, van der Zee J, Van Broeckhoven C, Le Ber I, Van Deerlin V, Suh E, Rohrer JD, Mead S, Graff C, Öijerstedt L, Pickering-Brown S, Rollinson S, Rossi G, Tagliavini F, Brooks WS, Dobson-Stone C, Halliday GM, Hodges JR, Piguet O, Binetti G, Benussi L, Ghidoni R, Nacmias B, Sorbi S, Bruni AC, Galimberti D, Scarpini E, Rainero I, Rubino E, Clarimon J, Lleó A, Ruiz A, Hernández I, Pastor P, Diez-Fairen M, Borroni B, Pasquier F, Deramecourt V, Lebouvier T, Perneczky R, Diehl-Schmid J, Grafman J, Huey ED, International FTD-Genomics Consortium (IFGC) , Mayeux R, Nalls MA, Hernandez D, Singleton A, Momeni P, Zeng Z, Hardy J, Robertson J, Zinman L, Rogaeva E


Converging Patterns of α-Synuclein Pathology in Multiple System Atrophy.

J Neuropathol Exp Neurol. 2018 Sep 07; Authors: Brettschneider J, Suh E, Robinson JL, Fang L, Lee EB, Irwin DJ, Grossman M, Van Deerlin VM, Lee VM, Trojanowski JQ


Mechanisms of Cell-to-Cell Transmission of Pathological Tau: A Review.

JAMA Neurol. 2018 Sep 04; Authors: Gibbons GS, Lee VMY, Trojanowski JQ


Validation of a Long-Read PCR Assay for Sensitive Detection and Sizing of C9orf72 Hexanucleotide Repeat Expansions.

J Mol Diagn. 2018 Aug 20; Authors: Suh E, Grando K, Van Deerlin VM


TFEB enhances astroglial uptake of extracellular tau species and reduces tau spreading.

J Exp Med. 2018 Aug 14; Authors: Martini-Stoica H, Cole AL, Swartzlander DB, Chen F, Wan YW, Bajaj L, Bader DA, Lee VMY, Trojanowski JQ, Liu Z, Sardiello M, Zheng H


Alzheimer's genetic risk is reduced in primary age-related tauopathy: a potential model of resistance?

Ann Clin Transl Neurol. 2018 Aug; Authors: McMillan CT, Lee EB, Jefferson-George K, Naj A, Van Deerlin VM, Trojanowski JQ, Wolk DA


Cerebrospinal fluid tau, Aβ, and sTREM2 in Former National Football League Players: Modeling the relationship between repetitive head impacts, microglial activation, and neurodegeneration.

Alzheimers Dement. 2018 Jul 23; Authors: Alosco ML, Tripodis Y, Fritts NG, Heslegrave A, Baugh CM, Conneely S, Mariani M, Martin BM, Frank S, Mez J, Stein TD, Cantu RC, McKee AC, Shaw LM, Trojanowski JQ, Blennow K, Zetterberg H, Stern RA


Non-Alzheimer's contributions to dementia and cognitive resilience in the 90+ Study.

Acta Neuropathol. 2018 June 18; Authors: Robinson JL, Corrada MM, Kovacs GG, Dominique M, Caswell C, Xie SX, Lee VM, Kawas CH, Trojanowski JQ


Sequential stages and distribution patterns of aging-related tay astrogliopathy (ARTAG) in the human brain.

Acta Neuropathol Commun. 2018 Jun 11; Authors: Kovacs GG, Xie SX, Robinson JL, Lee EB, Smith DH, Schuck T, Lee VM, Trojanowski JQ


Longitudinal structural gray matter and white matter MRI changes in presymptomatic progranulin mutation carriers.

Neuroimage Clin. 2018; Authors: Olm CA, McMillan CT, Irwin DJ, Van Deerlin VM, Cook PA, Gee JC, Grossman M


Induction of the Immunoproteasome Subunit Lmp7 Links Proteostasis and Immunity in α-Synuclein Aggregation Disorders.

EBioMedicine. 2018 May 11; Authors: Ugras S, Daniels MJ, Fazelinia H, Gould NS, Yocum AK, Luk KC, Luna E, Ding H, McKennan C, Seeholzer S, Martinez D, Evans P, Brown D, Duda JE, Ischiropoulos H


Design, synthesis and evaluation of photoactivatable derivatives of microtubule (MT)-active [1,2,4]triazolo[1,5-a]pyrimidines.

Bioorg Med Chem Lett. 2018 May 05; Authors: Oukoloff K, Kovalevich J, Cornec AS, Yao Y, Owyang ZA, James M, Trojanowski JQ, Lee VM, Smith AB, Brunden KR, Ballatore C


Lewy body-like alpha-synuclein inclusions trigger reactive microgliosis prior to nigral degeneration.

J Neuroinflammation. 2018 May 01; Authors: Duffy MF, Collier TJ, Patterson JR, Kemp CJ, Luk KC, Tansey MG, Paumier KL, Kanaan NM, Fischer LD, Polinski NK, Barth OL, Howe JW, Vaikath NN, Majbour NK, El-Agnaf OMA, Sortwell CE


Potential genetic modifiers of disease risk and age at onset in patients with frontotemporal lobar degeneration and GRN mutations: a genome-wide association study.

Lancet Neurol. 2018 Apr 30; Authors: Pottier C, Zhou X, Perkerson RB, Baker M, Jenkins GD, Serie DJ, Ghidoni R, Benussi L, Binetti G, López de Munain A, Zulaica M, Moreno F, Le Ber I, Pasquier F, Hannequin D, Sánchez-Valle R, Antonell A, Lladó A, Parsons TM, Finch NA, Finger EC, Lippa CF, Huey ED, Neumann M, Heutink P, Synofzik M, Wilke C, Rissman RA, Slawek J, Sitek E, Johannsen P, Nielsen JE, Ren Y, van Blitterswijk M, DeJesus-Hernandez M, Christopher E, Murray ME, Bieniek KF, Evers BM, Ferrari C, Rollinson S, Richardson A, Scarpini E, Fumagalli GG, Padovani A, Hardy J, Momeni P, Ferrari R, Frangipane F, Maletta R, Anfossi M, Gallo M, Petrucelli L, Suh E, Lopez OL, Wong TH, van Rooij JGJ, Seelaar H, Mead S, Caselli RJ, Reiman EM, Noel Sabbagh M, Kjolby M, Nykjaer A, Karydas AM, Boxer AL, Grinberg LT, Grafman J, Spina S, Oblak A, Mesulam MM, Weintraub S, Geula C, Hodges JR, Piguet O, Brooks WS, Irwin DJ, Trojanowski JQ, Lee EB, Josephs KA, Parisi JE, Ertekin-Taner N, Knopman DS, Nacmias B, Piaceri I, Bagnoli S, Sorbi S, Gearing M, Glass J, Beach TG, Black SE, Masellis M, Rogaeva E, Vonsattel JP, Honig LS, Kofler J, Bruni AC, Snowden J, Mann D, Pickering-Brown S, Diehl-Schmid J, Winkelmann J, Galimberti D, Graff C, Öijerstedt L, Troakes C, Al-Sarraj S, Cruchaga C, Cairns NJ, Rohrer JD, Halliday GM, Kwok JB, van Swieten JC, White CL, Ghetti B, Murell JR, Mackenzie IRA, Hsiung GR, Borroni B, Rossi G, Tagliavini F, Wszolek ZK, Petersen RC, Bigio EH, Grossman M, Van Deerlin VM, Seeley WW, Miller BL, Graff-Radford NR, Boeve BF, Dickson DW, Biernacka JM, Rademakers R


Cerebrospinal fluid neurogranin concentration in neurodegeneration: relation to clinical phenotypes and neuropathology.

Acta Neuropathol. 2018 Apr 26; Authors: Portelius E, Olsson B, Höglund K, Cullen NC, Kvartsberg H, Andreasson U, Zetterberg H, Sandelius Å, Shaw LM, Lee VMY, Irwin DJ, Grossman M, Weintraub D, Chen-Plotkin A, Wolk DA, McCluskey L, Elman L, McBride J, Toledo JB, Trojanowski JQ, Blennow K


Characterizing the human hippocampus in aging and Alzheimer's disease using a computational atlas derived from ex vivo MRI and histology.

Proc Natl Acad Sci U S A. 2018 Mar 28; Authors: Adler DH, Wisse LEM, Ittyerah R, Pluta JB, Ding SL, Xie L, Wang J, Kadivar S, Robinson JL, Schuck T, Trojanowski JQ, Grossman M, Detre JA, Elliott MA, Toledo JB, Liu W, Pickup S, Miller MI, Das SR, Wolk DA, Yushkevich PA


A 2-Step Cerebrospinal Algorithm for the Selection of Frontotemporal Lobar Degeneration Subtypes.

JAMA Neurol. 2018 Mar 19; Authors: Lleó A, Irwin DJ, Illán-Gala I, McMillan CT, Wolk DA, Lee EB, Van Deerlin VM, Shaw LM, Trojanowski JQ, Grossman M


The technical reliability and biotemporal stability of cerebrospinal fluid biomarkers for profiling multiple pathophysiologies in Alzheimer's disease.

PLoS One. 2018 Mar 05; Authors: Trombetta BA, Carlyle BC, Koenig AM, Shaw LM, Trojanowski JQ, Wolk DA, Locascio JJ, Arnold SE


Differential α-synuclein expression contributes to selective vulnerability of hippocampal neuron subpopulations to fibril-induced toxicity.

Acta Neuropathol. 2018 Mar 03; Authors: Luna E, Decker SC, Riddle DM, Caputo A, Zhang B, Cole T, Caswell C, Xie SX, Lee VMY, Luk KC


Measurements of auto-antibodies to α-synuclein in the serum and cerebral spinal fluids of patients with Parkinson's disease.

J Neurochem. 2018 Mar 03; Authors: Akhtar RS, Licata JP, Luk KC, Shaw LM, Trojanowski JQ, Lee VM


CSF biomarkers of Alzheimer's disease concord with amyloid-β PET and predict clinical progression: A study of fully automated immunoassays in BioFINDER and ADNI cohorts.

Alzheimers Dement. 2018 Feb 27; Authors: Hansson O, Seibyl J, Stomrud E, Zetterberg H, Trojanowski JQ, Bittner T, Lifke V, Corradini V, Eichenlaub U, Batrla R, Buck K, Zink K, Rabe C, Blennow K, Shaw LM, Swedish BioFINDER study group, Alzheimer's Disease Neuroimaging Initiative


CSF tau and amyloid-beta predict cerebral synucleinopathy in autopsied Lewy body disorders.

Neurology. 2018 Feb 21; Authors: Irwin DJ, Xie SX, Coughlin D, Nevler N, Akhtar RS, McMillan CT, Lee EB, Wolk DA, Weintraub D, Chen-Plotkin A, Duda JE, Spindler M, Siderowf A, Hurtig HI, Shaw LM, Grossman M, Trojanowski JQ


Microglia-mediated recovery from ALS-relevant motor neuron degeneration in a mouse model of TDP-43 proteinopathy.

Nat Neurosci. 2018 Feb 20; Authors: Spiller KJ, Restrepo CR, Khan T, Dominique MA, Fang TC, Canter RG, Roberts CJ, Miller KR, Ransohoff RM, Trojanowski JQ, Lee VM


Early vs late age at onset frontotemporal dementia and frontotemporal lobar degeneration.

Neurology. 2018 Feb 16; Authors: Seo SW, Thibodeau MP, Perry DC, Hua A, Sidhu M, Sible I, Vargas JNS, Gaus SE, Rabinovici GD, Rankin KD, Boxer AL, Kramer JH, Rosen HJ, Gorno-Tempini ML, Grinberg LT, Huang EJ, DeArmond SJ, Trojanowski JQ, Miller BL, Seeley WW


Best Practices for Generating and Using Alpha-Synuclein Pre-Formed Fibrils to Model Parkinson's Disease in Rodents.

J Parkinsons Dis. 2018 Jan 30; Authors: Polinski NK, Volpicelli-Daley LA, Sortwell CE, Luk KC, Cremades N, Gottler LM, Froula J, Duffy MF, Lee VM, Martinez TN, Dave KD


Induction of alpha-synuclein pathology in the enteric nervous system of the rat and non-human primate results in gastrointestinal dysmotility and transient CNS pathology.

Neurobiol Dis. 2018 Jan 13; Authors: Manfredsson FP, Luk KC, Benskey MJ, Guezer A, Garcia J, Kuhn NC, Sandoval IM, Patterson JR, O'Mara A, Yonkers R, Kordower JH


Investigating the genetic architecture of dementia with Lewy bodies: a two-stage genome-wide association study.

Lancet Neurol. 2018 Jan; Authors: Guerreiro R, Ross OA, Kun-Rodrigues C, Hernandez DG, Orme T, Eicher JD, Shepherd CE, Parkkinen L, Darwent L, Heckman MG, Scholz SW, Troncoso JC, Pletnikova O, Ansorge O, Clarimon J, Lleo A, Morenas-Rodriguez E, Clark L, Honig LS, Marder K, Lemstra A, Rogaeva E, St George-Hyslop P, Londos E, Zetterberg H, Barber I, Braae A, Brown K, Morgan K, Troakes C, Al-Sarraj S, Lashley T, Holton J, Compta Y, Van Deerlin V, Serrano GE, Beach TG, Lesage S, Galasko D, Masliah E, Santana I, Pastor P, Diez-Fairen M, Aguilar M, Tienari PJ, Myllykangas L, Oinas M, Revesz T, Lees A, Boeve BF, Petersen RC, Ferman TJ, Escott-Price V, Graff-Radford N, Cairns NJ, Morris JC, Pickering-Brown S, Mann D, Halliday GM, Hardy J, Trojanowski JQ, Dickson DW, Singleton A, Stone DJ, Bras J


A Longitudinal Study of Total and Phosphorylated α-Synuclein with Other Biomarkers in Cerebrospinal Fluid of Alzheimer's Disease and Mild Cognitive Impairment.

J Alzheimers Dis. 2018; Authors: Wang H, Stewart T, Toledo JB, Ginghina C, Tang L, Atik A, Aro P, Shaw LM, Trojanowski JQ, Galasko DR, Edland S, Jensen PH, Shi M, Zhang J, Alzheimer’s Disease Neuroimaging Initiative


Asymmetry of post-mortem neuropathology in behavioural-variant frontotemporal dementia.

Brain. 2017 Dec 08; Authors: Irwin DJ, McMillan CT, Xie SX, Rascovsky K, Van Deerlin VM, Coslett HB, Hamilton R, Aguirre G, Lee EB, Lee VMY, Trojanowski JQ, Grossman M


Cerebrospinal fluid, plasma, and saliva in the BioFIND study: Relationships among biomarkers and Parkinson's disease Features.

Mov Disord. 2017 Dec 04; Authors: Goldman JG, Andrews H, Amara A, Naito A, Alcalay RN, Shaw LM, Taylor P, Xie T, Tuite P, Henchcliffe C, Hogarth P, Frank S, Saint-Hilaire MH, Frasier M, Arnedo V, Reimer AN, Sutherland M, Swanson-Fischer C, Gwinn K, Kang UJ


Neocortical origin and progression of gray matter atrophy in nonamnestic Alzheimer's disease.

Neurobiol Aging. 2017 Nov 21; Authors: Phillips JS, Da Re F, Dratch L, Xie SX, Irwin DJ, McMillan CT, Vaishnavi SN, Ferrarese C, Lee EB, Shaw LM, Trojanowski JQ, Wolk DA, Grossman M


APOE, thought disorder, and SPARE-AD predict cognitive decline in established Parkinson's disease.

Mov Disord. 2017 Nov 23; Authors: Tropea TF, Xie SX, Rick J, Chahine LM, Dahodwala N, Doshi J, Davatzikos C, Shaw LM, Van Deerlin V, Trojanowski JQ, Weintraub D, Chen-Plotkin AS


Circulating brain-enriched microRNAs as novel biomarkers for detection and differentiation of neurodegenerative diseases.

Alzheimers Res Ther. 2017 Nov 09Authors: Sheinerman KS, Toledo JB, Tsivinsky VG, Irwin D, Grossman M, Weintraub D, Hurtig HI, Chen-Plotkin A, Wolk DA, McCluskey LF, Elman LB, Trojanowski JQ, Umansky SR


Longitudinal CSF biomarkers in patients with early Parkinson disease and healthy controls.

Neurology. 2017 Oct 13; Authors: Mollenhauer B, Caspell-Garcia CJ, Coffey CS, Taylor P, Shaw LM, Trojanowski JQ, Singleton A, Frasier M, Marek K, Galasko D, Parkinson's Progression Marker Initiative


Clinicopathological correlations in behavioural variant frontotemporal dementia.

Brain. 2017 Oct 06; Authors: Perry DC, Brown JA, Possin KL, Datta S, Trujillo A, Radke A, Karydas A, Kornak J, Sias AC, Rabinovici GD, Gorno-Tempini ML, Boxer AL, De May M, Rankin KP, Sturm VE, Lee SE, Matthews BR, Kao AW, Vossel KA, Tartaglia MC, Miller ZA, Seo SW, Sidhu M, Gaus SE, Nana AL, Vargas JNS, Hwang JL, Ossenkoppele R, Brown AB, Huang EJ, Coppola G, Rosen HJ, Geschwind D, Trojanowski JQ, Grinberg LT, Kramer JH, Miller BL, Seeley WW


Modeling Parkinson's disease pathology by combination of fibril seeds and α-synuclein overexpression in the rat brain.

Proc Natl Acad Sci U S A. 2017 Sep 12; Authors: Thakur P, Breger LS, Lundblad M, Wan OW, Mattsson B, Luk KC, Lee VMY, Trojanowski JQ, Björklund A


Neuron loss and degeneration in the progression of TDP-43 in frontotemporal lobar degeneration.

Acta Neuropathol Commun. 2017 Sep 06; Authors: Yousef A, Robinson JL, Irwin DJ, Byrne MD, Kwong LK, Lee EB, Xu Y, Xie SX, Rennert L, Suh E, Van Deerlin VM, Grossman M, Lee VM, Trojanowski JQ


Protein astrogliopathies in human neurodegenerative diseases and aging.

Brain Pathol. 2017 Sep; Authors: Kovacs GG, Lee VM, Trojanowski JQ


Excess α-synuclein compromises phagocytosis in iPSC-derived macrophages.

Sci Rep. 2017 Aug 21; Authors: Haenseler W, Zambon F, Lee H, Vowles J, Rinaldi F, Duggal G, Houlden H, Gwinn K, Wray S, Luk KC, Wade-Martins R, James WS, Cowley SA


Evaluation of Oxetan-3-ol, Thietan-3-ol, and Derivatives Thereof as Bioisosteres of the Carboxylic Acid Functional Group.

ACS Med Chem Lett. 2017 Aug 10; Authors: Lassalas P, Oukoloff K, Makani V, James M, Tran V, Yao Y, Huang L, Vijayendran K, Monti L, Trojanowski JQ, Lee VM, Kozlowski MC, Smith AB, Brunden KR, Ballatore C


Connexin-43 and Aquaporin-4 are markers of ARTAG-related astroglial response.

Neuropathol Appl Neurobiol. 2017 Jul 29; Authors: Kovacs GG, Yousef A, Kaindl S, Lee VM, Trojanowski JQ


Ante mortem CSF tau levels correlate with post mortem tau pathology in FTLD.

Ann Neurol. 2017 Jul 18; Authors: Irwin DJ, Lleó A, Xie SX, McMillan CT, Wolk D, Lee EB, Van Deerlin VM, Shaw LM, Trojanowski JQ, Grossman M


Clinically silent Alzheimer's and vascular pathologies influence brain networks supporting executive function in healthy older adults.

Neurobiol Aging. 2017 Jun 24; Authors: Gold BT, Brown CA, Hakun JG, Shaw LM, Trojanowski JQ, Smith CD


Phosphorylated neurofilament heavy chain: A biomarker of survival for C9ORF72-associated amyotrophic lateral sclerosis.

Ann Neurol. 2017 Jun 19; Authors: Gendron TF, Daughrity LM, Heckman MG, Diehl NN, Wuu J, Miller TM, Pastor P, Trojanowski JQ, Grossman M, Berry JD, Hu WT, Ratti A, Benatar M, Silani V, Glass JD, Floeter MK, Jeromin A, Boylan KB, Petrucelli L, C9ORF72 Neurofilament Study Group


Regional brain amyloid-β accumulation associates with domain-specific cognitive performance in Parkinson disease without dementia.

PLoS One. 2017 May 25; Authors: Akhtar RS, Xie SX, Chen YJ, Rick J, Gross RG, Nasrallah IM, Van Deerlin VM, Trojanowski JQ, Chen-Plotkin AS, Hurtig HI, Siderowf AD, Dubroff JG, Weintraub D


Clinical marker for Alzheimer disease pathology in logopenic primary progressive aphasia.

Neurology. 2017 May 17; Authors: Giannini LAA, Irwin DJ, McMillan CT, Ash S, Rascovsky K, Wolk DA, Van Deerlin VM, Lee EB, Trojanowski JQ, Grossman M


Novel conformation-selective alpha-synuclein antibodies raised against different in vitro fibril forms show distinct patterns of Lewy pathology in Parkinson's disease.

Neuropathol Appl Neurobiol. 2017 Apr 07; Authors: Covell DJ, Robinson JL, Akhtar RS, Grossman M, Weintraub D, Bucklin HM, Pitkin RM, Riddle D, Yousef A, Trojanowski JQ, Lee VM


Poly(GP) proteins are a useful pharmacodynamic marker for C9ORF72-associated amyotrophic lateral sclerosis.

Sci Transl Med. 2017 Mar 29;9(383): Authors: Gendron TF, Chew J, Stankowski JN, Hayes LR, Zhang YJ, Prudencio M, Carlomagno Y, Daughrity LM, Jansen-West K, Perkerson EA, O'Raw A, Cook C, Pregent L, Belzil V, van Blitterswijk M, Tabassian LJ, Lee CW, Yue M, Tong J, Song Y, Castanedes-Casey M, Rousseau L, Phillips V, Dickson DW, Rademakers R, Fryer JD, Rush BK, Pedraza O, Caputo AM, Desaro P, Palmucci C, Robertson A, Heckman MG, Diehl NN, Wiggs E, Tierney M, Braun L, Farren J, Lacomis D, Ladha S, Fournier CN, McCluskey LF, Elman LB, Toledo JB, McBride JD, Tiloca C, Morelli C, Poletti B, Solca F, Prelle A, Wuu J, Jockel-Balsarotti J, Rigo F, Ambrose C, Datta A, Yang W, Raitcheva D, Antognetti G, McCampbell A, Van Swieten JC, Miller BL, Boxer AL, Brown RH, Bowser R, Miller TM, Trojanowski JQ, Grossman M, Berry JD, Hu WT, Ratti A, Traynor BJ, Disney MD, Benatar M, Silani V, Glass JD, Floeter MK, Rothstein JD, Boylan KB, Petrucelli L


Evaluating the Patterns of Aging-Related Tau Astrogliopathy Unravels Novel Insights Into Brain Aging and Neurodegenerative Diseases.

J Neuropathol Exp Neurol. 2017 Mar 14; Authors: Kovacs GG, Robinson JL, Xie SX, Lee EB, Grossman M, Wolk DA, Irwin DJ, Weintraub D, Kim CF, Schuck T, Yousef A, Wagner ST, Suh E, Van Deerlin VM, Lee VM, Trojanowski JQ


Milder Alzheimer's disease pathology in heart failure and atrial fibrillation.

Alzheimers Dement. 2017 Feb 04; Authors: Sposato LA, Ruíz Vargas E, Riccio PM, Toledo JB, Trojanowski JQ, Kukull WA, Cipriano LE, Nucera A, Whitehead SN, Hachinski V


Expansion of the classification of FTLD-TDP: distinct pathology associated with rapidly progressive frontotemporal degeneration.

Acta Neuropathol. 2017 Jan 27; Authors: Lee EB, Porta S, Michael Baer G, Xu Y, Suh E, Kwong LK, Elman L, Grossman M, Lee VM, Irwin DJ, Van Deerlin VM, Trojanowski JQ


Non-Naturally Occurring Small Molecule Microtubule-Stabilizing Agents: A Potential Tactic for CNS-Directed Therapies.

ACS Chem Neurosci. 2017 Jan 18; Authors: Ballatore C, Brunden KR, Trojanowski JQ, Lee VM, Smith AB


Neuropathological and genetic correlates of survival and dementia onset in synucleinopathies: a retrospective analysis.

Lancet Neurol. 2017 Jan; Authors: Irwin DJ, Grossman M, Weintraub D, Hurtig HI, Duda JE, Xie SX, Lee EB, Van Deerlin VM, Lopez OL, Kofler JK, Nelson PT, Jicha GA, Woltjer R, Quinn JF, Kaye J, Leverenz JB, Tsuang D, Longfellow K, Yearout D, Kukull W, Keene CD, Montine TJ, Zabetian CP, Trojanowski JQ


Tauopathy with hippocampal 4-repeat tau immunoreactive spherical inclusions: a report of three cases.

Brain Pathol. 2016 Dec 26; Authors: Kovacs GG, Kwong LK, Grossman M, Irwin DJ, Lee EB, Robinson JL, Suh E, Van Deerlin VM, Lee VM, Trojanowski JQ


Altered microtubule dynamics in neurodegenerative disease: Therapeutic potential of microtubule-stabilizing drugs.

Neurobiol Dis. 2016 Dec 21; Authors: Brunden KR, Lee VM, Smith AB, Trojanowski JQ, Ballatore C


Longitudinal imaging reveals sub-hippocampal dynamics in glutamate levels associated with histopathologic events in a mouse model of tauopathy and healthy mice.

Hippocampus. 2016 Dec 20; Authors: Crescenzi R, DeBrosse C, Nanga RP, Byrne MD, Krishnamoorthy G, D'Aquilla K, Nath H, Morales KH, Iba M, Hariharan H, Lee VM, Detre JA, Reddy R


Hospital Care for mental health and substance abuse conditions in Parkinson's disease.

Mov Disord. 2016 Dec; Authors: Willis AW, Thibault DP, Schmidt PN, Dorsey ER, Weintraub D


Development, validation and application of a new fornix template for studies of aging and preclinical Alzheimer's disease.

Neuroimage Clin. 2016 Nov 26; Authors: Brown CA, Johnson NF, Anderson-Mooney AJ, Jicha GA, Shaw LM, Trojanowski JQ, Van Eldik LJ, Schmitt FA, Smith CD, Gold BT


Unique pathological tau conformers from Alzheimer's brains transmit tau pathology in nontransgenic mice.

J Exp Med. 2016 Oct 17; Authors: Guo JL, Narasimhan S, Changolkar L, He Z, Stieber A, Zhang B, Gathagan RJ, Iba M, McBride JD, Trojanowski JQ, Lee VM


Calcium dysregulation contributes to neurodegeneration in FTLD patient iPSC-derived neurons.

Sci Rep. 2016 Oct 10; Authors: Imamura K, Sahara N, Kanaan NM, Tsukita K, Kondo T, Kutoku Y, Ohsawa Y, Sunada Y, Kawakami K, Hotta A, Yawata S, Watanabe D, Hasegawa M, Trojanowski JQ, Lee VM, Suhara T, Higuchi M, Inoue H


Therapeutic strategies for the treatment of tauopathies: Hopes and challenges.

Alzheimers Dement. 2016 Oct; Authors: Khanna MR, Kovalevich J, Lee VM, Trojanowski JQ, Brunden KR


Evaluation of the brain-penetrant microtubule-stabilizing agent, dictyostatin, in the PS19 tau transgenic mouse model of tauopathy.

Acta Neuropathol Commun. 2016; Authors: Makani V, Zhang B, Han H, Yao Y, Lassalas P, Lou K, Paterson I, Lee VM, Trojanowski JQ, Ballatore C, Smith AB, Brunden KR


Progression of motor neuron disease is accelerated and the ability to recover is compromised with advanced age in rNLS8 mice.

Acta Neuropathol Commun. 2016; Authors: Spiller KJ, Restrepo CR, Khan T, Stieber AM, Kwong LK, Trojanowski JQ, Lee VM


Cognitive reserve in frontotemporal degeneration: Neuroanatomic and neuropsychological evidence.

Neurology. 2016 Sep 28; Authors: Placek K, Massimo L, Olm C, Ternes K, Firn K, Van Deerlin V, Lee EB, Trojanowski JQ, Lee VM, Irwin D, Grossman M, McMillan CT


Comparison of In Vivo and Ex Vivo MRI of the Human Hippocampal Formation in the Same Subjects.

Cereb Cortex. 2016 Sep 24; Authors: Wisse LE, Adler DH, Ittyerah R, Pluta JB, Robinson JL, Schuck T, Trojanowski JQ, Grossman M, Detre JA, Elliott MA, Toledo JB, Liu W, Pickup S, Das SR, Wolk DA, Yushkevich PA


Progression of alpha-synuclein pathology in multiple system atrophy of the cerebellar type.

Neuropathol Appl Neurobiol. 2016 Sep 22; Authors: Brettschneider J, Irwin DJ, Boluda S, Byrne MD, Fang L, Lee EB, Robinson JL, Suh E, Van Deerlin VM, Toledo JB, Grossman M, Hurtig H, Dengler R, Petri S, Lee VM, Trojanowski JQ


Analysis of C9orf72 repeat expansions in a large international cohort of dementia with Lewy bodies.

Neurobiol Aging. 2016 Sep 2; Authors: Kun-Rodrigues C, Ross OA, Orme T, Shepherd C, Parkkinen L, Darwent L, Hernandez D, Ansorge O, Clark LN, Honig LS, Marder K, Lemstra A, Scheltens P, van der Flier W, Louwersheimer E, Holstege H, Rogaeva E, St George-Hyslop P, Londos E, Zetterberg H, Barber I, Braae A, Brown K, Morgan K, Maetzler W, Berg D, Troakes C, Al-Sarraj S, Lashley T, Holton J, Compta Y, Van Deerlin V, Trojanowski JQ, Serrano GE, Beach TG, Clarimon J, Lleó A, Morenas-Rodríguez E, Lesage S, Galasko D, Masliah E, Santana I, Diez M, Pastor P, Tienari PJ, Myllykangas L, Oinas M, Revesz T, Lees A, Boeve BF, Petersen RC, Ferman TJ, Escott-Price V, Graff-Radford N, Cairns NJ, Morris JC, Stone DJ, Pickering-Brown S, Mann D, Dickson DW, Halliday GM, Singleton A, Guerreiro R, Bras J


Widespread transneuronal propagation of α-synucleinopathy triggered in olfactory bulb mimics prodromal Parkinson's disease.

J Exp Med. 2016 Aug 8; Authors: Rey NL, Steiner JA, Maroof N, Luk KC, Madaj Z, Trojanowski JQ, Lee VM, Brundin P


Amyloid-Beta Positron Emission Tomography Imaging of Alzheimer's Pathology in Parkinson's Disease Dementia.

Mov Disord Clin Pract. 2016 Jul-Aug; Authors: Akhtar RS, Xie SX, Brennan L, Pontecorvo MJ, Hurtig HI, Trojanowski JQ, Weintraub D, Siderowf AD


Genome-wide association analyses identify new risk variants and the genetic architecture of amyotrophic lateral sclerosis.

Nat Genet. 2016 Jul 25; Authors: van Rheenen W, Shatunov A, Dekker AM, McLaughlin RL, Diekstra FP, Pulit SL, van der Spek RA, Võsa U, de Jong S, Robinson MR, Yang J, Fogh I, van Doormaal PT, Tazelaar GH, Koppers M, Blokhuis AM, Sproviero W, Jones AR, Kenna KP, van Eijk KR, Harschnitz O, Schellevis RD, Brands WJ, Medic J, et al.


Selective Motor Neuron Resistance and Recovery in a New Inducible Mouse Model of TDP-43 Proteinopathy.

J Neurosci. 2016 Jul 20; Authors: Spiller KJ, Cheung CJ, Restrepo CR, Kwong LK, Stieber AM, Trojanowski JQ, Lee VM


Activation of HIPK2 Promotes ER Stress-Mediated Neurodegeneration in Amyotrophic Lateral Sclerosis.

Neuron. 2016 Jun 15; Authors: Lee S, Shang Y, Redmond SA, Urisman A, Tang AA, Li KH, Burlingame AL, Pak RA, Jovičić A, Gitler AD, Wang J, Gray NS, Seeley WW, Siddique T, Bigio EH, Lee VM, Trojanowski JQ, Chan JR, Huang EJ


Correction: Comparison of strategies for non-perturbing labeling of α-synuclein to study amyloidogenesis.

Org Biomol Chem. 2016 May 12; Authors: Haney CM, Wissner RF, Warner JB, Wang YJ, Ferrie JJ, Covell DJ, Karpowicz RJ, Lee VM, Petersson EJ


Relationship between APOE Genotype and Structural MRI Measures throughout Adulthood in the Study of Health in Pomerania Population-Based Cohort.

AJNR Am J Neuroradiol. 2016 May 12; Authors: Habes M, Toledo JB, Resnick SM, Doshi J, Van der Auwera S, Erus G, Janowitz D, Hegenscheid K, Homuth G, Völzke H, Hoffmann W, Grabe HJ, Davatzikos C


Features of Patients With Nonfluent/Agrammatic Primary Progressive Aphasia With Underlying Progressive Supranuclear Palsy Pathology or Corticobasal Degeneration.

JAMA Neurol. 2016 Apr 25; Authors: Santos-Santos MA, Mandelli ML, Binney RJ, Ogar J, Wilson SM, Henry ML, Hubbard HI, Meese M, Attygalle S, Rosenberg L, Pakvasa M, Trojanowski JQ, Grinberg LT, Rosen H, Boxer AL, Miller BL, Seeley WW, Gorno-Tempini ML


Defining and validating a short form Montreal Cognitive Assessment (s-MoCA) for use in neurodegenerative disease.

J Neurol Neurosurg Psychiatry. 2016 Apr 12; Authors: Roalf DR, Moore TM, Wolk DA, Arnold SE, Mechanic-Hamilton D, Rick J, Kabadi S, Ruparel K, Chen-Plotkin AS, Chahine LM, Dahodwala NA, Duda JE, Weintraub DA, Moberg PJ


Advanced brain aging: relationship with epidemiologic and genetic risk factors, and overlap with Alzheimer disease atrophy patterns.

Transl Psychiatry. 2016 April 5; Authors: Habes M, Janowitz D, Erus G, Toledo JB, Resnick SM, Doshi J, Van der Auwera S, Wittfeld K, Hegenscheid K, Hosten N, Biffar R, Homuth G, Völzke H, Grabe HJ, Hoffmann W, Davatzikos C


CSF biomarkers associated with disease heterogeneity in early Parkinson's disease: the Parkinson's Progression Markers Initiative study.

Acta Neuropathol. 2016 Mar 28; Authors: Kang JH, Mollenhauer B, Coffey CS, Toledo JB, Weintraub D, Galasko DR, Irwin DJ, Van Deerlin V, Chen-Plotkin AS, Caspell-Garcia C, Waligórska T, Taylor P, Shah N, Pan S, Zero P, Frasier M, Marek K, Kieburtz K, Jennings D, Tanner CM, Simuni T, Singleton A, Toga AW, Chowdhury S, Trojanowski JQ, Shaw LM, Parkinson’s Progression Marker Initiative


Characterization of brain-penetrant pyrimidine-containing molecules with differential microtubule-stabilizing activities developed as potential therapeutic agents for Alzheimer's disease and related tauopathies.

J Pharmacol Exp Ther. 2016 Mar 15; Authors: Kovalevich J, Cornec AS, Yao Y, James M, Crowe A, Lee VM, Trojanowski JQ, Smith AB, Ballatore C, Brunden KR


White matter hyperintensities and imaging patterns of brain ageing in the general population.

Brain. 2016 Feb 24; Authors: Habes M, Erus G, Toledo JB, Zhang T, Bryan N, Launer LJ, Rosseel Y, Janowitz D, Doshi J, Van der Auwera S, von Sarnowski B, Hegenscheid K, Hosten N, Homuth G, Völzke H, Schminke U, Hoffmann W, Grabe HJ, Davatzikos C


Evaluation of Cerebrospinal Fluid Assay Variability in Alzheimer's Disease.

J Alzheimers Dis. 2016 Feb 6; Authors: White MT, Shaw LM, Xie SX


An Alzheimer's Disease-Derived Biomarker Signature Identifies Parkinson's Disease Patients with Dementia.

PLoS One. 2016 Jan 26; Authors: Berlyand Y, Weintraub D, Xie SX, Mellis IA, Doshi J, Rick J, McBride J, Davatzikos C, Shaw LM, Hurtig H, Trojanowski JQ, Chen-Plotkin AS


Comparison of strategies for non-perturbing labeling of α-synuclein to study amyloidogenesis.

Org Biomol Chem. 2015 Dec 22; Authors: Haney CM, Wissner RF, Warner JB, Wang YJ, Ferrie JJ, J Covell D, Karpowicz RJ, Lee VM, James Petersson E


Inflammatory Eicosanoids Increase Amyloid Precursor Protein Expression via Activation of Multiple Neuronal Receptors.

Sci Rep. 2015 Dec 17; Authors: Herbst-Robinson KJ, Liu L, James M, Yao Y, Xie SX, Brunden KR


Aging-related tau astrogliopathy (ARTAG): harmonized evaluation strategy.

Acta Neuropathol. 2015 Dec 10; Authors: Kovacs GG, Ferrer I, Grinberg LT, Alafuzoff I, Attems J, Budka H, Cairns NJ, Crary JF, Duyckaerts C, Ghetti B, Halliday GM, Ironside JW, Love S, Mackenzie IR, Munoz DG, Murray ME, Nelson PT, Takahashi H, Trojanowski JQ, Ansorge O, Arzberger T, Baborie A, Beach TG, Bieniek KF, Bigio EH, Bodi I, Dugger BN, Feany M, Gelpi E, Gentleman SM, Giaccone G, Hatanpaa KJ, Heale R, Hof PR, Hofer M, Hortobágyi T, Jellinger K, Jicha GA, Ince P, Kofler J, Kövari E, Kril JJ, Mann DM, Matej R, McKee AC, McLean C, Milenkovic I, Montine TJ, Murayama S, Lee EB, Rahimi J, Rodriguez RD, Rozemüller A, Schneider JA, Schultz C, Seeley W, Seilhean D, Smith C, Tagliavini F, Takao M, Thal DR, Toledo JB, Tolnay M, Troncoso JC, Vinters HV, Weis S, Wharton SB, White CL, Wisniewski T, Woulfe JM, Yamada M, Dickson DW


Deep Clinical and Neuropathological Phenotyping of Pick's Disease.

Ann Neurol. 2015 Nov 19; Authors: Irwin DJ, Brettschneider J, McMillan CT, Cooper F, Olm C, Arnold SE, Van Deerlin VM, Seeley WW, Miller BL, Lee EB, Lee VM, Grossman M, Trojanowski JQ


Eye Movement Deficits Are Consistent with a Staging Model of pTDP-43 Pathology in Amyotrophic Lateral Sclerosis.

PLoS One. 2015 Nov 11; Authors: Gorges M, Müller HP, Lulé D, Del Tredici K, Brettschneider J, Keller J, Pfandl K, Ludolph AC, Kassubek J, Pinkhardt EH


Association of Cerebrospinal Fluid Neurofilament Light Concentration With Alzheimer Disease Progression.

JAMA Neurol. 2015 Nov 2; Authors: Zetterberg H, Skillbäck T, Mattsson N, Trojanowski JQ, Portelius E, Shaw LM, Weiner MW, Blennow K, Alzheimer’s Disease Neuroimaging Initiative


An insoluble frontotemporal lobar degeneration-associated TDP-43 C-terminal fragment causes neurodegeneration and hippocampus pathology in transgenic mice.

Hum Mol Genet. 2015 Oct 16; Authors: Walker AK, Tripathy K, Restrepo CR, Ge G, Xu Y, Kwong LK, Trojanowski JQ, Lee VM


Identifying amyloid pathology-related cerebrospinal fluid biomarkers for Alzheimer's disease in a multicohort study.

Alzheimers Dement (Amst). 2015 Sep 1; Authors: Leung YY, Toledo JB, Nefedov A, Polikar R, Raghavan N, Xie SX, Farnum M, Schultz T, Baek Y, Deerlin VV, Hu WT, Holtzman DM, Fagan AM, Perrin RJ, Grossman M, Soares HD, Kling MA, Mailman M, Arnold SE, Narayan VA, Lee VM, Shaw LM, Baker D, Wittenberg GM, Trojanowski JQ, Wang LS


Cerebrospinal fluid neurogranin: relation to cognition and neurodegeneration in Alzheimer's disease. 

Brain. 2015 Sep 15; Authors: Portelius E, Zetterberg H, Skillbäck T, Törnqvist U, Andreasson U, Trojanowski JQ, Weiner MW, Shaw LM, Mattsson N, Blennow K, Alzheimer’s Disease Neuroimaging Initiative


Semi-automated quantification of C9orf72 expansion size reveals inverse correlation between hexanucleotide repeat number and disease duration in frontotemporal degeneration. 

Acta Neuropathol. 2015 Sep; Authors: Suh E, Lee EB, Neal D, Wood EM, Toledo JB, Rennert L, Irwin DJ, McMillan CT, Krock B, Elman LB, McCluskey LF, Grossman M, Xie SX, Trojanowski JQ, Van Deerlin VM


Multisite assessment of NIA-AA guidelines for the neuropathologic evaluation of Alzheimer's disease.

Alzheimers Dement. 2015 Aug 29; Authors: Montine TJ, Monsell SE, Beach TG, Bigio EH, Bu Y, Cairns NJ, Frosch M, Henriksen J, Kofler J, Kukull WA, Lee EB, Nelson PT, Schantz AM, Schneider JA, Sonnen JA, Trojanowski JQ, Vinters HV, Zhou XH, Hyman BT


Diagnosis of Parkinson's disease on the basis of clinical and genetic classification: a population-based modelling study.

Lancet Neurol. 2015 Aug 10; Authors: Nalls MA, McLean CY, Rick J, Eberly S, Hutten SJ, Gwinn K, Sutherland M, Martinez M, Heutink P, Williams NM, Hardy J, Gasser T, Brice A, Price TR, Nicolas A, Keller MF, Molony C, Gibbs JR, Chen-Plotkin A, Suh E, Letson C, Fiandaca MS, Mapstone M, Federoff HJ, Noyce AJ, Morris H, Van Deerlin VM, Weintraub D, Zabetian C, Hernandez DG, Lesage S, Mullins M, Conley ED, Northover CA, Frasier M, Marek K, Day-Williams AG, Stone DJ, Ioannidis JP, Singleton AB, Parkinson's Disease Biomarkers Program and Parkinson's Progression Marker Initiative investigators*


Functional recovery in new mouse models of ALS/FTLD after clearance of pathological cytoplasmic TDP-43.

Acta Neuropathol. 2015 Jul 22; Authors: Walker AK, Spiller KJ, Ge G, Zheng A, Xu Y, Zhou M, Tripathy K, Kwong LK, Trojanowski JQ, Lee VM


Memory, executive, and multidomain subtle cognitive impairment: Clinical and biomarker findings.

Neurology. 2015 Jul 14; Authors: Toledo JB, Bjerke M, Chen K, Rozycki M, Jack CR, Weiner MW, Arnold SE, Reiman EM, Davatzikos C, Shaw LM, Trojanowski JQ, Alzheimer's Disease Neuroimaging Initiative


Common neuropathological features underlie distinct clinical presentations in three siblings with hereditary diffuse leukoencephalopathy with spheroids caused by CSF1R p.Arg782His.

Acta Neuropathol Commun. 2015 Jul 4; Authors: Robinson JL, Suh E, Wood EM, Lee EB, Coslett HB, Raible K, Lee VM, Trojanowski JQ, Van Deerlin VM


Role of brain infarcts in behavioral variant frontotemporal dementia: Clinicopathological characterization in the National Alzheimer's Coordinating Center database.

Neurobiol Aging. 2015 Oct, Epub 2015 Jul 3; Authors: Torralva T, Sposato LA, Riccio PM, Gleichgerrcht E, Roca M, Toledo JB, Trojanowski JQ, Kukull WA, Manes F, Hachinski V


Intrastriatal injection of pre-formed mouse α-synuclein fibrils into rats triggers α-synuclein pathology and bilateral nigrostriatal degeneration.

Neurobiol Dis. 2015 Jun 17; Authors: Paumier KL, Luk KC, Manfredsson FP, Kanaan NM, Lipton JW, Collier TJ, Steece-Collier K, Kemp CJ, Celano S, Schulz E, Sandoval IM, Fleming S, Dirr E, Polinski NK, Trojanowski JQ, Lee VM, Sortwell CE


Erratum to: Sequential distribution of pTDP-43 pathology in behavioral variant frontotemporal dementia (bvFTD).

Acta Neuropathol. 2015 Jun; Authors: Brettschneider J, Del Tredici K, Irwin DJ, Grossman M, Robinson JL, Toledo JB, Lee EB, Fang L, Van Deerlin VM, Ludolph AC, Lee VM, Braak H, Trojanowski JQ


Clinicopathological Study of Patients With C9ORF72-Associated Frontotemporal Dementia Presenting With Delusions.

J Geriatr Psychiatry Neurol. 2015 Jun; Authors: Shinagawa S, Naasan G, Karydas AM, Coppola G, Pribadi M, Seeley WW, Trojanowski JQ, Miller BL, Grinberg LT


Nonlinear Association Between Cerebrospinal Fluid and Florbetapir F-18 β-Amyloid Measures Across the Spectrum of Alzheimer Disease.

JAMA Neurol. 2015 May; Authors: Toledo JB, Bjerke M, Da X, Landau SM, Foster NL, Jagust W, Jack C, Weiner M, Davatzikos C, Shaw LM, Trojanowski JQ, Alzheimer’s Disease Neuroimaging Initiative Investigators


C9orf72 promoter hypermethylation is neuroprotective: Neuroimaging and neuropathologic evidence.

Neurology. 2015 Apr 21; Authors: McMillan CT, Russ J, Wood EM, Irwin DJ, Grossman M, McCluskey L, Elman L, Van Deerlin V, Lee EB


Frontotemporal lobar degeneration: defining phenotypic diversity through personalized medicine.

Acta Neuropathol. 2015 Apr; Authors: Irwin DJ, Cairns NJ, Grossman M, McMillan CT, Lee EB, Van Deerlin VM, Lee VM, Trojanowski JQ


PARK10 is a major locus for sporadic neuropathologically confirmed Parkinson disease.

Neurology. 2015 Mar 10; Authors: Beecham GW, Dickson DW, Scott WK, Martin ER, Schellenberg G, Nuytemans K, Larson EB, Buxbaum JD, Trojanowski JQ, Van Deerlin VM, Hurtig HI, Mash DC, Beach TG, Troncoso JC, Pletnikova O, Frosch MP, Ghetti B, Foroud TM, Honig LS, Marder K, Vonsattel JP, Goldman SM, Vinters HV, Ross OA, Wszolek ZK, Wang L, Dykxhoorn DM, Pericak-Vance MA, Montine TJ, Leverenz JB, Dawson TM, Vance JM


The TMEM106B locus and TDP-43 pathology in older persons without FTLD.

Neurology. 2015 Mar 3; Authors: Yu L, De Jager PL, Yang J, Trojanowski JQ, Bennett DA, Schneider JA


Differential induction and spread of tau pathology in young PS19 tau transgenic mice following intracerebral injections of pathological tau from Alzheimer's disease or corticobasal degeneration brains.

Acta Neuropathol. 2015 Feb; Authors: Boluda S, Iba M, Zhang B, Raible KM, Lee VM, Trojanowski JQ


Intracerebral injection of preformed synthetic tau fibrils initiates widespread tauopathy and neuronal loss in the brains of tau transgenic mice.

Neurobiol Dis. 2015 Jan; Authors: Peeraer E, Bottelbergs A, Van Kolen K, Stancu IC, Vasconcelos B, Mahieu M, Duytschaever H, Ver Donck L, Torremans A, Sluydts E, Van Acker N, Kemp JA, Mercken M, Brunden KR, Trojanowski JQ, Dewachter I, Lee VM, Moechars D