TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration

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hTAF(II)68, a novel RNA/ssDNA‐binding protein with homology to the pro‐oncoproteins TLS/FUS and EWS is associated with both TFIID and RNA polymerase II.

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TLS (FUS) binds RNA in vivo and engages in nucleo-cytoplasmic shuttling

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TDP43 is a human low molecular weight neurofilament (hNFL) mRNA-binding protein

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SC35 autoregulates its expression by promoting splicing events that destabilize its mRNAs

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The multifunctional FUS, EWS and TAF15 proto-oncoproteins show cell type-specific expression patterns and involvement in cell spreading and stress response

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TDP‐43, the signature protein of FTLD‐U, is a neuronal activity‐responsive factor

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TDP-43 in the Ubiquitin Pathology of Frontotemporal Dementia With VCP Gene Mutations

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Structural insights into TDP-43 in nucleic-acid binding and domain interactions

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The RNA Binding Protein TLS Is Translocated to Dendritic Spines by mGluR5 Activation and Regulates Spine Morphology

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Pathological Heterogeneity of Frontotemporal Lobar Degeneration with Ubiquitin-Positive Inclusions Delineated by Ubiquitin Immunohistochemistry and Novel Monoclonal Antibodies

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DCTN1 mutations in Perry syndrome

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Evidence of Multisystem Disorder in Whole-Brain Map of Pathological TDP-43 in Amyotrophic Lateral Sclerosis

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Structural diversity and functional implications of the eukaryotic TDP gene family

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TDP-43 accumulation in inclusion body myopathy muscle suggests a common pathogenic mechanism with frontotemporal dementia

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Depletion of TDP‐43 affects Drosophila motoneurons terminal synapsis and locomotive behavior

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Protein composition of human prespliceosomes isolated by a tobramycin affinity-selection method

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Ubiquitinated pathological lesions in frontotemporal lobar degeneration contain the TAR DNA-binding protein, TDP-43

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Fus deficiency in mice results in defective B-lymphocyte development and activation, high levels of chromosomal instability and perinatal death

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TDP-43 immunoreactivity in neuronal inclusions in familial amyotrophic lateral sclerosis with or without SOD1 gene mutation

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Truncation and pathogenic mutations facilitate the formation of intracellular aggregates of TDP-43

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LINE-1 ORF1 Protein Localizes in Stress Granules with Other RNA-Binding Proteins, Including Components of RNA Interference RNA-Induced Silencing Complex

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Colocalization of Transactivation-Responsive DNA-Binding Protein 43 and Huntingtin in Inclusions of Huntington Disease

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The TET Family of Proteins: Functions and Roles in Disease

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A Drosophila model for TDP-43 proteinopathy

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HDAC6–p97/VCP controlled polyubiquitin chain turnover

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RNA and RNA Binding Proteins Participate in Early Stages of Cell Spreading through Spreading Initiation Centers

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A novel effector domain from the RNA-binding protein TLS or EWS is required for oncogenic transformation by CHOP.Preprint

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EWS, but Not EWS-FLI-1, Is Associated with Both TFIID and RNA Polymerase II: Interactions between Two Members of the TET Family, EWS and hTAF II 68, and Subunits of TFIID and RNA Polymerase II Complexes

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TDP‐43, a neuro‐pathosignature factor, is essential for early mouse embryogenesis

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FUS mutations in amyotrophic lateral sclerosis: clinical, pathological, neurophysiological and genetic analysis

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Abnormal TDP-43 immunoreactivity in AD modifies clinicopathologic and radiologic phenotype

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FUS pathology in basophilic inclusion body disease

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TLS facilitates transport of mRNA encoding an actin-stabilizing protein to dendritic spines

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Male sterility and enhanced radiation sensitivity in TLS−/− mice

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TDP-43 is deposited in the Guam parkinsonism-dementia complex brains

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Mutation within TARDBP leads to Frontotemporal Dementia without motor neuron disease

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TDP-43-Positive White Matter Pathology in Frontotemporal Lobar Degeneration With Ubiquitin-Positive Inclusions

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TDP-43 regulates retinoblastoma protein phosphorylation through the repression of cyclin-dependent kinase 6 expression

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Depletion of TDP 43 overrides the need for exonic and intronic splicing enhancers in the human apoA-II gene

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Knockdown of transactive response DNA‐binding protein (TDP‐43) downregulates histone deacetylase 6

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Functional mapping of the interaction between TDP-43 and hnRNP A2 in vivo

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Genetic contribution of FUS to frontotemporal lobar degeneration

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Rapidly progressive aphasic dementia and motor neuron disease

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Divergent patterns of cytosolic TDP-43 and neuronal progranulin expression following axotomy: Implications for TDP-43 in the physiological response to neuronal injury

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High frequency ofTARDBPgene mutations in Italian patients with amyotrophic lateral sclerosis

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Transgenic mice expressing mutant forms VCP/p97 recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and bone

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Sarcoplasmic redistribution of nuclear TDP‐43 in inclusion body myositis

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Transgenic Rat Model of Neurodegeneration Caused by Mutation in the TDP Gene

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TARDBP variation associated with frontotemporal dementia, supranuclear gaze palsy, and chorea

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Contribution of TARDBP mutations to sporadic amyotrophic lateral sclerosis

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Pathological TDP-43 in parkinsonism–dementia complex and amyotrophic lateral sclerosis of Guam

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Higher order arrangement of the eukaryotic nuclear bodies

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Identification of an RNA Binding Specificity for the Potential Splicing Factor TLS

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Sporadic amyotrophic lateral sclerosis: two pathological patterns shown by analysis of distribution of TDP-43-immunoreactive neuronal and glial cytoplasmic inclusions

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Myosin-Va Facilitates the Accumulation of mRNA/Protein Complex in Dendritic Spines

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Human 75-kDa DNA-pairing Protein Is Identical to the Pro-oncoprotein TLS/FUS and Is Able to Promote D-loop Formation

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Mutations of FUS gene in sporadic amyotrophic lateral sclerosis

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Rapamycin Rescues TDP-43 Mislocalization and the Associated Low Molecular Mass Neurofilament Instability

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Molecular Neuropathology of TDP-43 Proteinopathies

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Missense Mutations in the Progranulin Gene Linked to Frontotemporal Lobar Degeneration with Ubiquitin-immunoreactive Inclusions Reduce Progranulin Production and Secretion

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Phenotype of Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Dementia with ALS (FTD/ALS) Associated with the GGGGCC Repeat Expansion in C9ORF72 (c9FTD/ALS) (S05.003)

Boylan, K. A., DeJesus-Hernandez, M., Rush, B., Desaro, P., Johnston, A., Kryston, T., Rutherford, N., Baker, M., Wszolek, Z., Dickson, D. W., et al. (2012) · Neurology · doi:10.1212/wnl.78.1_meetingabstracts.s05.003

Nutritional and metabolic status at diagnosis and survival of patients with Amyotrophic Lateral Sclerosis (ALS) and frontotemporal dementia (FTD) compared to patients with ALS alone

Jésus, P., Sourisseau, H., Lautrette, G., et al. (2020) · HAL (Le Centre pour la Communication Scientifique Directe)

Half of the patients with amyotrophic lateral sclerosis after ventilation had apparent frontotemporal lobar atrophy: A Quantitative survey of 92 patients by CT imaging

Sugano, K., Yoshino, H., Nakazato, T., et al. (2017) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2017.08.2007

Current Concepts in the Classification and Diagnosis of Frontotemporal Lobar Degenerations: A Practical Approach

Rohan, Z., & Matěj, R. (2013) · Archives of Pathology & Laboratory Medicine · doi:10.5858/arpa.2012-0510-rs · Cited by 15 · see citing works

Genetic analysis of matrin 3 gene in French amyotrophic lateral sclerosis patients and frontotemporal lobar degeneration with amyotrophic lateral sclerosis patients

Millecamps, S., de Septenville, A., Teyssou, E., et al. (2014) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2014.07.016 · Cited by 42 · see citing works