To date, publications whose work was supported by Bluefield funding include:

2023

• Jiskoot LC et al. The Benson Complex Figure Test detects deficits in visuoconstruction and visual memory in symptomatic familial frontotemporal dementia: A GENFI study. J Neurol Sci. 2023 Mar 15;446:120590. 

• Samra K et al. Motor symptoms in genetic frontotemporal dementia: developing a new module for clinical rating scales. J Neurol. 2023 Mar;270(3):1466-1477. 

• Marsan E et al. Astroglial toxicity promotes synaptic degeneration in the thalamocortical circuit in frontotemporal dementia with GRN mutations. J Clin Invest. 2023 Mar 15;133(6):e164919

• Premi E et al. Early neurotransmitters changes in prodromal frontotemporal dementia: A GENFI study. Neurobiol Dis. 2023 Mar 8;179:106068. 

• Smith DM et al. Targeting nonsense-mediated RNA decay does not increase progranulin levels in the Grn R493X mouse model of frontotemporal dementia. PLoS One. 2023 Mar 9;18(3):e0282822. 

• Kashyap SN et al. Preclinical Interventions in Mouse Models of Frontotemporal Dementia Due to Progranulin Mutations. Neurotherapeutics. 2023 Feb 13. 

• Samra K et al. Neuropsychiatric symptoms in genetic frontotemporal dementia: developing a new module for Clinical Rating Scales. J Neurol Neurosurg Psychiatry. 2023 Jan 10:jnnp-2022-330152. 

2022

• Woollacott IOC et al. CSF glial markers are elevated in a subset of patients with genetic frontotemporal dementia. Ann Clin Transl Neurol. 2022 Nov;9(11):1764-1777. 
• Zetterberg H et al. The role of neurofilament light in genetic frontotemporal lobar degeneration. Brain Commun. 2022 Nov 26;5(1):fcac310. 
• Poos JM et al. Longitudinal Brain Atrophy Rates in Presymptomatic Carriers of Genetic Frontotemporal Dementia. Neurology. 2022 Oct 26;99(24):e2661–71. 
• Staffaroni AM et al. Temporal order of clinical and biomarker changes in familial frontotemporal dementia. Nat Med. 2022 Oct;28(10):2194-2206. 
• Boland S et al. Deficiency of the frontotemporal dementia gene GRN results in gangliosidosis. Nat Commun. 2022 Oct 7;13(1):5924. 
• van der Ende E et al. Elevated CSF and plasma complement proteins in genetic frontotemporal dementia: results from the GENFI study. J Neuroinflammation. 2022 Sep 5;19(1):217.
• Sogorb-Esteve A et al. Differential impairment of cerebrospinal fluid synaptic biomarkers in the genetic forms of frontotemporal dementia. Alzheimers Res Ther. 2022 Aug 31;14(1):118. 
• Gerrits E et al. Neurovascular dysfunction in GRN-associated frontotemporal dementia identified by single-nucleus RNA sequencing of human cerebral cortex. Nat Neurosci. 2022 Aug;25(8):1034-1048. 
• Hashimoto K et al. Neuroimmune dysfunction in frontotemporal dementia: Insights from progranulin and C9orf72 deficiency. Curr Opin Neurobiol. 2022 Jul 2;76:102599. 
• Nelson A et al. The CBI-R detects early behavioural impairment in genetic frontotemporal dementia. Ann Clin Transl Neurol. 2022 May;9(5):644-658. 
• Boeve BF et al. Advances and controversies in frontotemporal dementia: diagnosis, biomarkers, and therapeutic considerations. Lancet Neurol. 2022 Mar;21(3):258-272.
• Foster PH et al. Examining empathy deficits across familial forms of frontotemporal dementia within the GENFI cohort. Cortex. 2022 Feb 9;150:12-28.
• Devireddy S, and Ferguson SM. Efficient progranulin exit from the ER requires its interaction with prosaposin, a Surf4 cargo. J Cell Biol. 2022 Feb 7;221(2):e202104044.
• Zhang T et al. Differential regulation of progranulin derived granulin peptides. Mol Neurodegener. 2022 Feb 4;17(1):15. 
• Peakman G et al. Comparison of clinical rating scales in genetic frontotemporal dementia within the GENFI cohort. J Neurol Neurosurg Psychiatry. 2022 Feb;93(2):158-168. 
• Poos JM et al. Cognitive composites for genetic frontotemporal dementia: GENFI-Cog. Alzheimers Res Ther. 2022 Jan 19;14(1):10.
• Wilke C et al. Stratifying the Presymptomatic Phase of Genetic Frontotemporal Dementia by Serum NfL and pNfH: A Longitudinal Multicentre Study. Ann Neurol. 2022 Jan;91(1):33-47.
• Du H et al. Regulation of lysosomal trafficking of progranulin by sortilin and prosaposin. Brain Commun. 2022 Jan 4;4(1):fcab310.

2021

• van der Ende, EL et al. A data-driven disease progression model of fluid biomarkers in genetic frontotemporal dementia, Brain, 2021;, awab382, https://doi.org/10.1093/brain/awab382
• Bergström, S et al. A panel of CSF proteins separates genetic frontotemporal dementia from presymptomatic mutation carriers: a GENFI study. Mol Neurodegeneration 16, 79 (2021). https://doi.org/10.1186/s13024-021-00499-4
• Ljubenkov, PA et al. Effect of the Histone Deacetylase Inhibitor FRM-0334 on Progranulin Levels in Patients With Progranulin Gene Haploinsufficiency: A Randomized Clinical Trial. JAMA Netw Open. 2021;4(9):e2125584
• Öijerstedt L et al. Practice effects in genetic frontotemporal dementia and at-risk individuals: a GENFI study. J Neurol Neurosurg Psychiatry. 2021 Aug 18:jnnp-2021-327005. 
• Root J et al. Lysosome dysfunction as a cause of neurodegenerative diseases: Lessons from frontotemporal dementia and amyotrophic lateral sclerosis. Neurobiol Dis. 2021 Jul;154:105360
• Rojas, JC et al. Plasma Neurofilament Light for Prediction of Disease Progression in Familial Frontotemporal Lobar Degeneration. Neurology 2021.10.1212
• Poos JM et al. Impairment of episodic memory in genetic frontotemporal dementia: A GENFI study. Alzheimers Dement (Amst). 2021 May 13;13(1):e12185. 
• Feng T et al. Physiological and pathological functions of TMEM106B: a gene associated with brain aging and multiple brain disorders. Acta Neuropathol. 2021 Jan 1. 
• Swift IJ et al. Fluid biomarkers in frontotemporal dementia: past, present and future. J Neurol Neurosurg Psychiatry. 2021 Feb;92(2):204-215.
• Panman JL et al. Modelling the cascade of biomarker changes in GRN-related frontotemporal dementia. J Neurol Neurosurg Psychiatry. 2021 Jan 15:jnnp-2020-323541. 

2020

• Borrego-Écija S et al. Disease-related cortical thinning in presymptomatic granulin mutation carriers. Neuroimage Clin. 2020 Dec 29;29:102540. 
• Huang M et al. Network analysis of the progranulin-deficient mouse brain proteome reveals pathogenic mechanisms shared in human frontotemporal dementia caused by GRN mutations. acta neuropathol commun. 2020 8, 163.

• Russell LL et al. Social cognition impairment in genetic frontotemporal dementia within the GENFI cohort. Cortex. 2020 Dec;133:384-398. 

• Häkkinen S et al. Neuroimaging in genetic frontotemporal dementia and amyotrophic lateral sclerosis. Neurobiol Dis. 2020 Nov;145:105063. 

• Feng T et al. Loss of TMEM106B and PGRN leads to severe lysosomal abnormalities and neurodegeneration in mice. EMBO Rep. 2020 Oct 5;21(10):e50219. 

• Arrant AE et al. Elevated levels of extracellular vesicles in progranulin-deficient mice and FTD-GRN Patients. Ann Clin Transl Neurol. 2020 Nov 16;7(12):2433–49. 

• Zhou X et al. Loss of TMEM106B leads to myelination deficits: implications for frontotemporal dementia treatment strategies. Brain 2020;143(6):1905–19.
• Olney NT et al. Clinical and volumetric changes with increasing functional impairment in familial frontotemporal lobar degeneration. Alzheimers Dement 2020;16:49–59.
• Pasquini L et al. Salience Network Atrophy Links Neuron Type-Specific Pathobiology to Loss of Empathy in Frontotemporal Dementia. Cerebral Cortex 2020;1225:59. 
• van der Ende EL et al. Neuronal pentraxin 2: a synapse-derived CSF biomarker in genetic frontotemporal dementia. Journal of Neurology, Neurosurgery & Psychiatry 2020;91(6):612–21. 
• Ramos EM et al. Genetic screening of a large series of North American sporadic and familial frontotemporal dementia cases. Alzheimers Dement 2020;16(1):118–30. 
• Elia LP et al. Approaches to develop therapeutics to treat frontotemporal dementia. Neuropharmacology. 2020 Apr;166:107948. 
• Miyagawa T et al. Utility of the global CDR® plus NACC FTLD rating and development of scoring rules: Data from the ARTFL/LEFFTDS Consortium. Alzheimers Dement 2020;16(1):106–17. 
• Boeve B et al. The longitudinal evaluation of familial frontotemporal dementia subjects protocol: Framework and methodology. Alzheimers Dement 2020;16:22–36.
• Staffaroni AM et al. Assessment of executive function declines in presymptomatic and mildly symptomatic familial frontotemporal dementia: NIH-EXAMINER as a potential clinical trial endpoint. Alzheimers Dement 2020;16:11–21.
• Boxer AL et al. New directions in clinical trials for frontotemporal lobar degeneration: Methods and outcome measures. Alzheimers Dement 2020;16:131–43. 
• Narendra DP et al. Coupling APEX labeling to imaging mass spectrometry of single organelles reveals heterogeneity in lysosomal protein turnover. JCB 2020;219(1):436. 

2019

• Arrant AE et al. Impaired β-glucocerebrosidase activity and processing in frontotemporal dementia due to progranulin mutations. Acta Neuropathol Commun 2019;7(1):218–17. 

• van der Ende EL et al. Novel CSF biomarkers in genetic frontotemporal dementia identified by proteomics. Ann Clin Transl Neurol 2019;6(4):698–707. 

• Moore KM et al. Age at symptom onset and death and disease duration in genetic frontotemporal dementia: an international retrospective cohort study. Lancet Neurol 2019:145-156.
• Roczniak-Ferguson A & Ferguson SM. Pleiotropic requirements for human TDP-43 in the regulation of cell and organelle homeostasis. Life Sci Alliance 2019;2(5):e201900358.

• Kornak J et al. Nonlinear Z-score modeling for improved detection of cognitive abnormality. Alzheimers Dement 2019;11:797–808. 

• Chen Q et al. Tracking white matter degeneration in asymptomatic and symptomatic MAPT mutation carriers. Neurobiol Aging 2019;83:54–62.

• Ljubenkov PA et al. Peripheral Innate Immune Activation Correlates With Disease Severity in GRN Haploinsufficiency. Front Neur 2019;10:1004. 

• Tavares T. P. et al. Ventricular volume expansion in presymptomatic genetic frontotemporal dementia. Neurology (2019).

• Moreno-Yruela C. et al. Kinetic Tuning of HDAC Inhibitors Affords Potent Inducers of Progranulin Expression. ACS Chem Neurosci (2019).

• Butler V. J. et al. Age- and stress-associated C. elegans granulins impair lysosomal function and induce a compensatory HLH-30/TFEB transcriptional response. PLoS Genet 2019;15(8):e1008295.

• Lee, S. E. et al. Thalamo-cortical network hyperconnectivity in preclinical progranulin mutation carriers. Neuroimage Clin 22, 101751 (2019).

• Jiskoot, L. C. et al. Longitudinal multimodal MRI as prognostic and diagnostic biomarker in presymptomatic familial frontotemporal dementia. Brain 142, 193–208 (2019).

• Pottier, C. et al. Genome-wide analyses as part of the international FTLD-TDP whole-genome sequencing consortium reveals novel disease risk factors and increases support for immune dysfunction in FTLD. Acta Neuropathol 1–21 (2019). 

• Butler, V. J. et al. Progranulin Stimulates the in vitro Maturation of pro-Cathepsin D at Acidic pH. J Mol Biol (2019). 

• Elia, L. P. et al. Genetic Regulation of Neuronal Progranulin Reveals a Critical Role for the Autophagy-Lysosome Pathway. J Neurosci 3498–17 (2019). 

2018

• Xian, X. et al. Reversal of ApoE4-induced recycling block as a novel prevention approach for Alzheimer's disease. eLife 7, 7779 (2018).

• Arrant, A. E. et al. Reduction of microglial progranulin does not exacerbate pathology or behavioral deficits in neuronal progranulin-insufficient mice. Neurobiology of Disease 124, 152–162 (2018).

• Zetterberg, H., et al. Fluid biomarkers for frontotemporal dementias. Neuropathol Appl Neurobiol (2018).

• Hofmann, J. W. et al. RNA Binding Proteins and the Pathogenesis of Frontotemporal Lobar Degeneration. Annu Rev Pathol 14, 421059372 (2018).

• Bonham, L. W. et al. Protein network analysis reveals selectively vulnerable regions and biological processes in FTD. Neurol Genet 4, e266 (2018).

• Nguyen, A. D. et al. Progranulin in the hematopoietic compartment protects mice from atherosclerosis. Atherosclerosis 277, 145–154 (2018).

• Geier, E. G. et al. Rare variants in the neuronal ceroid lipofuscinosis gene MFSD8 are candidate risk factors for frontotemporal dementia. Acta Neuropathol 526, 68–18 (2018).

• Woollacott, I. O. C. et al. Cerebrospinal fluid soluble TREM2 levels in frontotemporal dementia differ by genetic and pathological subgroup. Alzheimers Res Ther 10, 79 (2018).

• Woollacott, I. O. C. et al. Pathological correlates of white matter hyperintensities in a case of progranulin mutation associated frontotemporal dementia. Neurocase 2, 1–9 (2018).

• Gan, L. et al. Converging pathways in neurodegeneration, from genetics to mechanisms. Nat Neurosci 21, 1300–1309 (2018).

• Ferguson, S. M. Neuronal lysosomes. Neuroscience Letters (2018). 

• Sturm, V. E. et al. Network Architecture Underlying Basal Autonomic Outflow: Evidence from Frontotemporal Dementia. J. Neurosci 38, 8943–8955 (2018).

• Arrant, A. E. et al. Partial Tmem106b reduction does not correct abnormalities due to progranulin haploinsufficiency. Mol Neurodegeneration 13, 32 (2018).

• Nicholson, A. M. et al. Loss of Tmem106b is unable to ameliorate frontotemporal dementia-like phenotypes in an AAV mouse model of C9ORF72-repeat induced toxicity. Acta Neuropathol Commun 6, 42 (2018).

• Wang, C. et al. Endo-lysosomal dysfunction: a converging mechanism in neurodegenerative diseases. Cur Op Neurobiol 48, 52–58 (2018).

• Jiskoot, L. C. et al. Presymptomatic white matter integrity loss in familial frontotemporal dementia in the GENFI cohort: A cross-sectional diffusion tensor imaging study. Ann Clin Transl Neurol 5, 1025–1036 (2018)

• Fumagalli, G. G. et al. Distinct patterns of brain atrophy in Genetic Frontotemporal Dementia Initiative (GENFI) cohort revealed by visual rating scales. Alzheimers Res Ther 10, 46 (2018).

• Cooper, Y. A. et al. Progranulin levels in blood in Alzheimer's disease and mild cognitive impairment. Ann Clin Transl Neurol 5, 616–629 (2018).

• Pottier et al. 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).

• Nguyen et al. Murine knockin model for progranulin-deficient frontotemporal dementia with nonsense-mediated mRNA decay. PNAS. (2018).

• Arrant et al. Progranulin Gene Therapy Improves Lysosomal Dysfunction and Microglial Pathology Associated with Frontotemporal Dementia and Neuronal Ceroid Lipofuscinosis. J. Neurosci (2018).

• Galimberti, D. et al. Progranulin plasma levels predict the presence of GRN mutations in asymptomatic subjects and do not correlate with brain atrophy: results from the GENFI study. Neurobio Aging (2018).  

 2017

• Evers BM et al. Lipidomic and Transcriptomic Basis of Lysosomal Dysfunction in Progranulin Deficiency. Cell Rep. 2017 Sep 12;20(11):2565-2574. 

• Perry, D. C. et al. Clinicopathological correlations in behavioural variant frontotemporal dementia. Brain (2017).

• Seeley, W. W. Mapping Neurodegenerative Disease Onset and Progression. Cold Spring Harb Perspect Biol. (2017).

• Holler, C. J. et al. Intracellular Proteolysis of Progranulin Generates Stable, Lysosomal Granulins that Are Haploinsufficient in Patients with Frontotemporal Dementia Caused by GRN Mutations. eNeuro (2017).

• She, A. et al. Selectivity and Kinetic Requirements of HDAC Inhibitors as Progranulin Enhancers for Treating Frontotemporal Dementia. Cell Chem Biol 24, 892–906.e5 (2017).

• van de Sluis, B. et al. News on the molecular regulation and function of hepatic low-density lipoprotein receptor and LDLR-related protein 1. Curr Opin Lipidol 28, 241–247 (2017)

• Meeter, L. H. et al. Imaging and fluid biomarkers in frontotemporal dementia. Nat Rev Neurol 13, 406–419 (2017).

• Zhou, X. et al. Impaired prosaposin lysosomal trafficking in frontotemporal lobar degeneration due to progranulin mutations. Nat Commun 8, 15277 (2017).

• Cheng, C. et al. Highly Expandable Human iPS Cell-Derived Neural Progenitor Cells (NPC) and Neurons for Central Nervous System Disease Modeling and High-Throughput Screening. Curr Protoc Hum Genet 92, 21.8.1–21.8.21 (2017).

• Arrant, A. E. et al. Restoring neuronal progranulin reverses deficits in a mouse model of frontotemporal dementia. Brain (2017).

• Ward, M. E. et al. Individuals with progranulin haploinsufficiency exhibit features of neuronal ceroid lipofuscinosis. Sci Trans Med (2017).

• Krabbe, G. et al. Microglial NFkB-TNFalpha hyperactivation induces obsessive-compulsive behavior in mouse models of progranulin-deficient frontotemporal dementia. PNAS 114, 5029–5034 (2017).

• Wasser, C. R. & Herz, J. Reelin: Neurodevelopmental Architect and Homeostatic Regulator of Excitatory Synapses. JBC 292, 1330–1338 (2017).

• Kao, A. W. et al. Progranulin, lysosomal regulation and neurodegenerative disease. Nat Rev Neurosci 31, 1245 (2017).

• Mason, A. R. et al. The Receptor-Interacting Serine/Threonine Protein Kinase 1 (Ripk1) Regulates Progranulin Levels. JBC (2017).

• Spinelli, E. G. et al. Typical and atypical pathology in primary progressive aphasia variants. Ann Neurol. (2017).

• Amick, J. & Ferguson, S. M. C9orf72: At the intersection of lysosome cell biology and neurodegenerative disease. Traffic (2017). 

2016

• Miller, Z. A. et al. Increased prevalence of autoimmune disease within C9 and FTD/MND cohorts: Completing the picture. Neurol Neuroimmunol Neuroinflamm 3, e301 (2016).

• Amick, J., et al. C9orf72 binds SMCR8, localizes to lysosomes and regulates mTORC1 signaling. Mol. Biol. Cell (2016).

• Nicholson, A. M. & Rademakers, R. What we know about TMEM106B in neurodegeneration. Acta Neuropathol 1–13 (2016).

• Ranasinghe, K. G. et al. Distinct Subtypes of Behavioral Variant Frontotemporal Dementia Based on Patterns of Network Degeneration. JAMA Neurol (2016).

• Santos-Santos, M. et al. Features of Patients With Nonfluent/Agrammatic Primary Progressive Aphasia With Underlying Progressive Supranuclear Palsy Pathology or Corticobasal Degeneration. JAMA Neurol. 73:733-742 (2016).

• Nicholson, A. M. et al. Prosaposin is a regulator of progranulin levels and oligomerization. Nat Commun. 7, 11992 (2016).

• Arrant, A. et al. Progranulin haploinsufficiency causes biphasic social dominance abnormalities in the tube test. Genes Brain Behav (2016).
• Lui, H et al. Progranulin Deficiency Promotes Circuit-Specific Synaptic Pruning by Microglia via Complement Activation. Cell 165, 921–935 (2016).
• Tsai, R. M. & Boxer, A. L. Therapy and clinical trials in frontotemporal dementia: past, present, and future. J Neurochem. (2016).
• Wasser, C. R. & Herz, J. Splicing therapeutics for Alzheimer's disease. EMBO Mol Med 8, 308–310 (2016).
• Pottier, C et al. Genetics of FTLD: Overview and what else we can expect from genetic studies. J Neurochem (2016)
• Almeida, S. et al. Suberoylanilide hydroxamic acid increases progranulin production in iPSC-derived cortical neurons of frontotemporal dementia patients. Neurobiol Aging (2016).

2015

• Minami, S. S. et al. Reducing inflammation and rescuing FTD-related behavioral deficits in progranulin-deficient mice with ?7 nicotinic acetylcholine receptor agonists. Biochemical Pharmacology (2015).
• Lee, S. & Huang, E. J. Modeling ALS and FTD with iPSC-derived neurons. Brain Research (2015)
• Arrant, A. E. et al. Effects of Exercise on Progranulin Levels and Gliosis in Progranulin-Insufficient Mice. eNeuro 2, (2015).
• Kao, P. F. et al. Detection of TDP-43 oligomers in frontotemporal lobar degeneration-TDP. Ann Neurol. (2015).
• Ossenkoppele, R. et al. The behavioural/dysexecutive variant of Alzheimer's disease: clinical, neuroimaging and pathological features. Brain awv191 (2015).
• Gowrishankar, S. et al. Massive accumulation of luminal protease-deficient axonal lysosomes at Alzheimer's disease amyloid plaques. PNAS (2015).
• Salazar, D. A. et al. The Progranulin Cleavage Products, Granulins, Exacerbate TDP-43 Toxicity and Increase TDP-43 Levels. J. Neurosci 35, 9315–9328 (2015).
• Ferguson, S. M. Beyond indigestion: emerging roles for lysosome-based signaling in human disease. Current Opinion in Cell Biology 35, 59–68 (2015).
• Villeneuve, S. et al. Existing Pittsburgh Compound-B positron emission tomography thresholds are too high: statistical and pathological evaluation. Brain 138, 2020–2033 (2015).
• Cho, S.-H. et al. SIRT1 deficiency in microglia contributes to cognitive decline in aging and neurodegeneration via epigenetic regulation of IL-1?. J. Neurosci 35, 807–818 (2015).

2014

• Minami, S. S. et al. Progranulin protects against amyloid ? deposition and toxicity in Alzheimer's disease mouse models. Nat Med (2014).
• Sephton, C. F. et al. Activity-dependent FUS dysregulation disrupts synaptic homeostasis. Proceedings of the National Academy of Sciences 111, E4769–78 (2014).
• Lee, S. E. et al. Altered network connectivity in frontotemporal dementia with C9orf72 hexanucleotide repeat expansion. Brain awu248 (2014).
• Warmus, B. A. et al. Tau-mediated NMDA receptor impairment underlies dysfunction of a selectively vulnerable network in a mouse model of frontotemporal dementia. J Neurosci 34, 16482–16495 (2014).
• van Blitterswijk, M. et al. Genetic modifiers in carriers of repeat expansions in the C9ORF72 gene. Mol Neurodegeneration 9, 38 (2014).
• Ward, M. E. et al. Early retinal neurodegeneration and impaired Ran-mediated nuclear import of TDP-43 in progranulin-deficient FTLD. Journal of Experimental Medicine 177, 311 (2014)
• Ferrari, R. et al. Frontotemporal dementia and its subtypes: a genome-wide association study. Lancet Neurol 13, 686–699 (2014).
• Nicholson, A. M. et al. Progranulin protein levels are differently regulated in plasma and CSF. Neurology (2014)
• van Blitterswijk et al. TMEM106B protects C9ORF72 expansion carriers against frontotemporal dementia. Acta Neuropathol (2014) 

2013

• Scherling et al. Cerebrospinal fluid neurofilament concentration reflects disease severity in frontotemporal degeneration. Ann Neurol. (2013) pp.
• Lee et al. Targeted manipulation of the sortilin-progranulin axis rescues progranulin haploinsufficiency. Human Molecular Genetics (2013) pp.
• Perry and Miller. Frontotemporal dementia. Semin Neurol (2013) vol. 33 (4) pp. 336-41
• Ravenscroft et al. Mutations in protein N-arginine methyltransferases are not the cause of FTLD-FUS. Neurobiol Aging (2013) vol. 34 (9) pp. 2235.e11-3
• van Blitterswijk et al. C9ORF72 repeat expansions in cases with previously identified pathogenic mutations. Neurology (2013) vol. 81 (15) pp. 1332-41
• Judy et al. A shift to organismal stress resistance in programmed cell death mutants. PLoS Genet (2013) vol. 9 (9) pp. e1003714
• Nguyen et al. Progranulin: at the interface of neurodegenerative and metabolic diseases. Trends Endocrinol Metab (2013)
• Almeida et al. Modeling key pathological features of frontotemporal dementia with C9ORF72 repeat expansion in iPSC-derived human neurons. Acta Neuropathol (2013)
• Nicholson et al. TMEM106B p.T185S regulates TMEM106B protein levels: implications for frontotemporal dementia. J Neurochem (2013)
• Chen et al. Progranulin Does Not Bind Tumor Necrosis Factor (TNF) Receptors and Is Not a Direct Regulator of TNF-Dependent Signaling or Bioactivity in Immune or Neuronal Cells. J Neurosci (2013) vol. 33 (21) pp. 9202-9213
• Miller et al. TDP-43 frontotemporal lobar degeneration and autoimmune disease. J Neurol Neurosurg Psychiatr (2013)
• Filiano et al. Dissociation of Frontotemporal Dementia-Related Deficits and Neuroinflammation in Progranulin Haploinsufficient Mice. J Neurosci  vol. 33 (12) pp. 5352-536 (2013)
• Nguyen et al. Secreted progranulin is a homodimer and is not a component of high-density lipoproteins (HDL). J Biol Chem (2013)
• Halabi et al. Patterns of striatal degeneration in frontotemporal dementia. Alzheimer disease and associated disorders vol. 27 (1) pp. 74-83 (2013)

2012

• Blitterswijk et al. How do C9ORF72 repeat expansions cause amyotrophic lateral sclerosis and frontotemporal dementia: can we learn from other noncoding repeat expansion disorders? Current Opinion in Neurology. vol. 25 (6) pp. 689-700 (2012)
• Henry et al. Neuropsychological, behavioral, and anatomical evolution in right temporal variant frontotemporal dementia: A longitudinal and post-mortem single case analysis. Neurocase (2012)
• Rutherford et al. TMEM106B risk variant is implicated in the pathologic presentation of Alzheimer disease. Neurology vol. 79 (7) pp. 717-8 (2012)
• Roberson, E. Mouse models of frontotemporal dementia. Ann Neurol. vol. 72 (6) pp. 837-49 (2012)
• Armakola et al. Inhibition of RNA lariat debranching enzyme suppresses TDP-43 toxicity in ALS disease models. Nature Genetics vol 44(12):1302-9 (2012)
• Almeida et al. Induced Pluripotent Stem Cell Models of Progranulin-Deficient Frontotemporal Dementia Uncover Specific Reversible Neuronal Defects. Cell Rep vol 2(4):789-98 (2012)
• Martens et al. Progranulin deficiency promotes neuroinflammation and neuron loss in toxin-induced CNS injury. JCI. vol 122(11):3955-9 (2012)
• Sephton et al. TDP-43 in central nervous system development and function: clues to TDP-43-associated neurodegeneration. J. Biol Chem vol. 393 (7) pp. 589-94 (2012)
• Seeley et al. Frontotemporal Dementia: What Can the Behavioral Variant Teach Us about Human Brain Organization? Neuroscientist vol. 18 (4) pp. 373-85 (2012)
• Rademakers R et al. Advances in understanding the molecular basis of frontotemporal dementia. Nat Rev Neurol vol. 8 (8) pp. 423-34 (2012)
• Sha et al. Frontotemporal dementia due to C9ORF72 mutations: Clinical and imaging features. Neurology vol. 79 (10) pp. 1002-11 (2012)
• Zhou et al.  Predicting regional neurodegeneration from the healthy brain functional connectome. Neuron vol. 73 (6) pp. 1216-27 (2012)
• Rutherford et al. TMEM106B risk variant is implicated in the pathologic presentation of Alzheimer disease. Neurology vol. 79 (7) pp. 717-8 (2012)
• Dries et al. Extracting ?-amyloid from Alzheimer's disease. Proc Natl Acad Sci USA vol. 109 (9) pp. 3199-200 (2012)
• Cenik et al. Progranulin: a proteolytically processed protein at the crossroads of inflammation and neurodegeneration. J Biol Chem vol.  287 (39) pp. 32298-32306 (2012)
• Dewey et al. TDP-43 aggregation in neurodegeneration: Are stress granules the key? Brain Res (2012).

2011

• Rabinovici et al. Amyloid vs FDG-PET in the differential diagnosis of AD and FTLD. Neurology (2011).
• Gozal et al. Aberrant Septin 11 is Associated with Sporadic Frontotemporal Lobar Degeneration. Mol Neurodegener (2011).
• Lee et al. Clinical characterization of bvFTD due to FUS neuropathology. Neurocase (2011).
• Kocerha et al. Altered microRNA expressionin frontotemporal lobar degeneration with TDP-43 pathology caused by progranulin mutations. BMC Genomics (2011).
• Almeida et al. Progranulin, a glycoprotein deficient in frontotemporal dementia, is a novel substrate of several protein disultide isomerase family proteins. PLoS One (2011).
• Wexler et al. Genome-wide analysis of the Wnt1 transcriptional network implicates neurodegenerative pathways. Science Signaling (2011).
• Rosen et al. Functional Genomic Analyses Identify Pathways Dysregulated by Progranulin Deficiency. Neuron (2011).
• Nicholson et al. Human genetics as a tool to identify progranulin regulators. J Mol Neurosci (2011).
• Cho et al. CSCR1 modulates microglial activation and protects against plaque-independent cognitive deficits in a mouse model of Alzheimers disease. J Biol Chem (2011).
• Finch et al. TMEM106B regulates progranulin leels and the penetrance of frontotemporal lobar degeneration in GRN mutation carriers. Neurology (2011).
• Cenik et al. SAHA (Vorinostat) upregulates progranulin transcription: A rational therapeutic approach to frontotemporal dementia. JBC (2011).
• Kao et al. A neurodegenerative disease mutation that accelerates the clearance of apoptotic cells. Proc Natl Acad Sci USA (2011).

2010

• Rohrer et al. TDP-43 subtypes are associated with distinct atrophy patterns in frontotemporal dementia. Neurology (2010).
• Dewey et al. TDP-43 is directed to stress granules by sorbitol, a novel physiological osmotic and oxidative stressor. Mol Cell Biol (2010).
• Carrasquillo et al. Genome-wide screen identifies rs646776 near sortilin as a regulator of progranulin levels in human plasma. Am J Human Genet (2010).

• Sephton et al. Identification of neuronal RNA targets of TDP-43-containing Ribonucleoprotein complexes. J Biol Chem (2010).

• Chen et al. ApoE4 reduces glutamate receptor function and synaptic plasticity by selectively impairing ApoE receptor recycling. Proc Natl Acad Sci USA.  (2010). 107(26):12011-16
• Forster et al. Emerging topics in Reelin function. European Journal of Neuroscience, (2010). Vol. 31: 1511–18.
• Sephton et al. TDP-43 is a developmentally regulated protein essential for early embryonic development. J Biol Chem (2010). 285(9):6826-34. 
• Jiao et al. MicroRNA-29b regulates the expression level of human progranulin, a secreted glycoprotein implicated in frontotemporal dementia. PLoS ONE (2010). vol. 5 (5) pp. e10551.
• Barmada et al. Cytoplasmic mislocalization of TDP-43 is toxic to neurons and enhanced by a mutation associate with familial amyotrophic lateral sclerosis. J. Neurosci (2010). 13:639-649
• Barmada et al. Pathogenic TARDBP mutations in amyotrophic lateral sclerosis and frontotemporal dementia: disease associated pathways. Neursci. Rev (2010). 21: 251-272

2008-9

• Daub et al. High-content screening of primary neurons: ready for prime time. Curr Opin Neurobiol (2009). 19(5):537-43.
• Coppola et al. Gene Expression Study on Peripheral Blood Identifies Progranulin Mutations. Annals of Neurology (2008). 64(1):92-96