Altered Tau Isoform Ratio Caused by Loss of FUS and SFPQ Function Leads to FTLD-like Phenotypes.

[1]  Timothy A. Miller,et al.  Increased 4R-Tau Induces Pathological Changes in a Human-Tau Mouse Model , 2016, Neuron.

[2]  H. Okano,et al.  Establishment of In Vitro FUS-Associated Familial Amyotrophic Lateral Sclerosis Model Using Human Induced Pluripotent Stem Cells , 2016, Stem cell reports.

[3]  Xiang-Dong Fu,et al.  Toxic gain of function from mutant FUS protein is crucial to trigger cell autonomous motor neuron loss , 2016, The EMBO journal.

[4]  J. Tapia,et al.  ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function , 2016, Nature Communications.

[5]  S. Mead,et al.  Review: An update on clinical, genetic and pathological aspects of frontotemporal lobar degenerations , 2015, Neuropathology and applied neurobiology.

[6]  G. Sobue,et al.  FUS regulates AMPA receptor function and FTLD/ALS-associated behaviour via GluA1 mRNA stabilization , 2015, Nature Communications.

[7]  T. Hashikawa,et al.  FUS/TLS deficiency causes behavioral and pathological abnormalities distinct from amyotrophic lateral sclerosis , 2015, Acta neuropathologica communications.

[8]  Giovanni Coppola,et al.  Familial Behavioral Variant Frontotemporal Dementia Associated With Astrocyte-Predominant Tauopathy , 2015, Journal of neuropathology and experimental neurology.

[9]  Gang Chen,et al.  Interaction of amyotrophic lateral sclerosis/frontotemporal lobar degeneration–associated fused-in-sarcoma with proteins involved in metabolic and protein degradation pathways , 2015, Neurobiology of Aging.

[10]  Gene W. Yeo,et al.  ALS-causative mutations in FUS/TLS confer gain- and loss-of-function by altered association with SMN and U1-snRNP , 2015, Nature Communications.

[11]  G. Sobue,et al.  Lower motor neuron involvement in TAR DNA-binding protein of 43 kDa-related frontotemporal lobar degeneration and amyotrophic lateral sclerosis. , 2014, JAMA neurology.

[12]  Adriano Chiò,et al.  State of play in amyotrophic lateral sclerosis genetics , 2013, Nature Neuroscience.

[13]  G. Sobue,et al.  Amyotrophic lateral sclerosis: an update on recent genetic insights , 2013, Journal of Neurology.

[14]  G. Sobue,et al.  FUS-regulated region- and cell-type-specific transcriptome is associated with cell selectivity in ALS/FTLD , 2013, Scientific Reports.

[15]  Michel Goedert,et al.  Tau pathology and neurodegeneration , 2013, The Lancet Neurology.

[16]  Wim Robberecht,et al.  The changing scene of amyotrophic lateral sclerosis , 2013, Nature Reviews Neuroscience.

[17]  Fumiaki Tanaka,et al.  Spliceosome integrity is defective in the motor neuron diseases ALS and SMA , 2013, EMBO molecular medicine.

[18]  C. Broeckhoven,et al.  The molecular basis of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum. , 2012 .

[19]  D. Dickson,et al.  Characteristics of TBS-extractable hyperphosphorylated tau species: aggregation intermediates in rTg4510 mouse brain. , 2012, Journal of Alzheimer's disease : JAD.

[20]  Stephanie C Huelga,et al.  Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs , 2012, Nature Neuroscience.

[21]  Nejc Haberman,et al.  Widespread binding of FUS along nascent RNA regulates alternative splicing in the brain , 2012, Scientific Reports.

[22]  C. Haass,et al.  Loss of fused in sarcoma (FUS) promotes pathological Tau splicing , 2012, EMBO reports.

[23]  Kinji Ohno,et al.  Position-dependent FUS-RNA interactions regulate alternative splicing events and transcriptions , 2012, Scientific Reports.

[24]  J. Ávila,et al.  Tau Protein and Adult Hippocampal Neurogenesis , 2012, Front. Neurosci..

[25]  N. Shneider,et al.  The ALS-associated proteins FUS and TDP-43 function together to affect Drosophila locomotion and life span. , 2011, The Journal of clinical investigation.

[26]  Jane Y. Wu,et al.  PSF Suppresses Tau Exon 10 Inclusion by Interacting with a Stem-Loop Structure Downstream of Exon 10 , 2011, Journal of Molecular Neuroscience.

[27]  E. Brustein,et al.  FUS and TARDBP but Not SOD1 Interact in Genetic Models of Amyotrophic Lateral Sclerosis , 2011, PLoS genetics.

[28]  Dennis W. Dickson,et al.  Neuropathology of Frontotemporal Lobar Degeneration-Tau (FTLD-Tau) , 2011, Journal of Molecular Neuroscience.

[29]  J. Hodges,et al.  Neuropathological background of phenotypical variability in frontotemporal dementia , 2011, Acta Neuropathologica.

[30]  A. Eisen,et al.  Pathological heterogeneity in amyotrophic lateral sclerosis with FUS mutations: two distinct patterns correlating with disease severity and mutation , 2011, Acta Neuropathologica.

[31]  E. Aquilanti,et al.  Intracellular localization and splicing regulation of FUS/TLS are variably affected by amyotrophic lateral sclerosis-linked mutations , 2010, Nucleic acids research.

[32]  Nick C Fox,et al.  Clinical, genetic and pathological heterogeneity of frontotemporal dementia: a review , 2010, Journal of Neurology, Neurosurgery & Psychiatry.

[33]  E. Mugnaini,et al.  FUS‐immunoreactive inclusions are a common feature in sporadic and non‐SOD1 familial amyotrophic lateral sclerosis , 2010, Annals of neurology.

[34]  H. Kretzschmar,et al.  A new subtype of frontotemporal lobar degeneration with FUS pathology. , 2009, Brain : a journal of neurology.

[35]  D. Cleveland,et al.  Rethinking ALS: The FUS about TDP-43 , 2009, Cell.

[36]  John Q. Trojanowski,et al.  Amyotrophic lateral sclerosis, frontotemporal dementia and beyond: the TDP-43 diseases , 2009, Journal of Neurology.

[37]  Xun Hu,et al.  Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6 , 2009, Science.

[38]  G. Schellenberg,et al.  Tau isoform regulation is region‐ and cell‐specific in mouse brain , 2008, The Journal of comparative neurology.

[39]  Ryoichiro Kageyama,et al.  Roles of continuous neurogenesis in the structural and functional integrity of the adult forebrain , 2008, Nature Neuroscience.

[40]  G. Schellenberg,et al.  Arginine/Serine-rich Protein Interaction Domain-dependent Modulation of a Tau Exon 10 Splicing Enhancer , 2006, Journal of Biological Chemistry.

[41]  F Barkhof,et al.  Hippocampal atrophy on MRI in frontotemporal lobar degeneration and Alzheimer’s disease , 2005, Journal of Neurology, Neurosurgery & Psychiatry.

[42]  T. Misawa,et al.  Adeno-Associated Viral Vector-Mediated Expression of Endostatin Inhibits Tumor Growth and Metastasis in an Orthotropic Pancreatic Cancer Model in Hamsters , 2004, Cancer Research.

[43]  N. Kawai,et al.  Adeno-associated viral vector-mediated gene therapy of ischemia-induced neuronal death. , 2002, Methods in enzymology.

[44]  J L Haines,et al.  Supporting Online Material Materials and Methods Figs. S1 to S7 Tables S1 to S4 References Mutations in the Fus/tls Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis , 2022 .