Unraveling the Pathways to Neuronal Homeostasis and Disease: Mechanistic Insights into the Role of RNA-Binding Proteins and Associated Factors

Dynamic duo – FMRP and TDP-43: Regulating common targets, causing different diseases

(2018) Diana Ferro et al.   BRAIN RESEARCH

A Mild PUM1 Mutation Is Associated with Adult-Onset Ataxia, whereas Haploinsufficiency Causes Developmental Delay and Seizures

(2018) Vincenzo A. Gennarino et al.   CELL

Neuron-Specific HuR-Deficient Mice Spontaneously Develop Motor Neuron Disease

(2018) Kevin Sun et al.   JOURNAL OF IMMUNOLOGY

The TIA1 RNA-Binding Protein Family Regulates EIF2AK2-Mediated Stress Response and Cell Cycle Progression

(2018) Cindy Meyer et al.   MOLECULAR CELL

TDP-43 gains function due to perturbed autoregulation in a Tardbp knock-in mouse model of ALS-FTD

(2018) Matthew A. White et al.   NATURE NEUROSCIENCE

TDP-43 pathology disrupts nuclear pore complexes and nucleocytoplasmic transport in ALS/FTD

(2018) Ching-Chieh Chou et al.   NATURE NEUROSCIENCE

A brave new world of RNA-binding proteins

(2018) Matthias W. Hentze et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

A Translational Repression Complex in Developing Mammalian Neural Stem Cells that Regulates Neuronal Specification

(2018) Siraj K. Zahr et al.   NEURON

Sumoylation regulates FMRP-mediated dendritic spine elimination and maturation

(2018) Anouar Khayachi et al.   Nature Communications

Elavl3 is essential for the maintenance of Purkinje neuron axons

(2018) Yuki Ogawa et al.   Scientific Reports

Accumulation of poly(A) RNA in nuclear granules enriched in Sam68 in motor neurons from the SMNΔ7 mouse model of SMA

(2018) J. Oriol Narcís et al.   Scientific Reports

Modeling Fragile X Syndrome in Drosophila

(2018) Małgorzata Drozd et al.   Frontiers in Molecular Neuroscience

Of Men and Mice: Modeling the Fragile X Syndrome

(2018) Regina Dahlhaus   Frontiers in Molecular Neuroscience

Post-transcriptional regulation of mouse neurogenesis by Pumilio proteins

(2017) Meng Zhang et al.   GENES & DEVELOPMENT

Genetic mutations in RNA-binding proteins and their roles in ALS

(2017) Katannya Kapeli et al.   HUMAN GENETICS

Axonal ribosomes and mRNAs associate with fragile X granules in adult rodent and human brains

(2017) Michael R. Akins et al.   HUMAN MOLECULAR GENETICS

Transactive response DNA-binding protein 43 (TDP-43) regulates alternative splicing of tau exon 10: Implications for the pathogenesis of tauopathies

(2017) Jianlan Gu et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

HuD and the Survival Motor Neuron Protein Interact in Motoneurons and Are Essential for Motoneuron Development, Function, and mRNA Regulation

(2017) Thi Hao le et al.   JOURNAL OF NEUROSCIENCE

T-Cell Intracellular Antigens and Hu Antigen R Antagonistically Modulate Mitochondrial Activity and Dynamics by Regulating Optic Atrophy 1 Gene Expression

(2017) Isabel Carrascoso et al.   MOLECULAR AND CELLULAR BIOLOGY

Reducing the RNA binding protein TIA1 protects against tau-mediated neurodegeneration in vivo

(2017) Daniel J. Apicco et al.   NATURE NEUROSCIENCE

Nusinersen, an antisense oligonucleotide drug for spinal muscular atrophy

(2017) David R Corey   NATURE NEUROSCIENCE

TIA1 Mutations in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Promote Phase Separation and Alter Stress Granule Dynamics

(2017) Ian R. Mackenzie et al.   NEURON

A novel role of fragile X mental retardation protein in pre-mRNA alternative splicing through RNA-binding protein 14

(2017) Lin-Tao Zhou et al.   NEUROSCIENCE

TDP-43 suppresses tau expression via promoting its mRNA instability

(2017) Jianlan Gu et al.   NUCLEIC ACIDS RESEARCH

Amyotrophic lateral sclerosis-linked mutations increase the viscosity of liquid-like TDP-43 RNP granules in neurons

(2017) Pallavi P. Gopal et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

SMN deficiency in severe models of spinal muscular atrophy causes widespread intron retention and DNA damage

(2017) Mohini Jangi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Combinatorial control of messenger RNAs by Pumilio, Nanos and Brain Tumor Proteins

(2017) René M. Arvola et al.   RNA Biology

Absence of the Fragile X Mental Retardation Protein results in defects of RNA editing of neuronal mRNAs in mouse

(2017) Alice Filippini et al.   RNA Biology

RNA localization is a key determinant of neurite-enriched proteome

(2017) Alessandra Zappulo et al.   Nature Communications

The Survival of Motor Neuron Protein Acts as a Molecular Chaperone for mRNP Assembly

(2017) Paul G. Donlin-Asp et al.   Cell Reports

Loss of FMRP Impaired Hippocampal Long-Term Plasticity and Spatial Learning in Rats

(2017) Yonglu Tian et al.   Frontiers in Molecular Neuroscience

nELAV Proteins Alteration in Alzheimer's Disease Brain: A Novel Putative Target for Amyloid-β Reverberating on AβPP Processing

(2017) Marialaura Amadio et al.   JOURNAL OF ALZHEIMERS DISEASE

Neuronal ELAVL proteins utilize AUF-1 as a co-partner to induce neuron-specific alternative splicing of APP

(2017) Apostolia Fragkouli et al.   Scientific Reports

ZBP1 phosphorylation at serine 181 regulates its dendritic transport and the development of dendritic trees of hippocampal neurons

(2017) Anna S. Urbanska et al.   Scientific Reports

Dynamic Axonal Translation in Developing and Mature Visual Circuits

(2016) Toshiaki Shigeoka et al.   CELL

The new (dis)order in RNA regulation

(2016) Aino I. Järvelin et al.   Cell Communication and Signaling

T-cell-restricted intracellular antigen 1 facilitates mitochondrial fragmentation by enhancing the expression of mitochondrial fission factor

(2016) Hyosun Tak et al.   CELL DEATH AND DIFFERENTIATION

Expression of Sam68 Associates with Neuronal Apoptosis and Reactive Astrocytes After Spinal Cord Injury

(2016) Xinlei Chen et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

Synapse Dysfunction of Layer V Pyramidal Neurons Precedes Neurodegeneration in a Mouse Model of TDP-43 Proteinopathies

(2016) Emily E. Handley et al.   CEREBRAL CORTEX

TDP-43 binds and transports G-quadruplex-containing mRNAs into neurites for local translation

(2016) Akira Ishiguro et al.   GENES TO CELLS

Extensive cryptic splicing upon loss of RBM17 and TDP43 in neurodegeneration models

(2016) Qiumin Tan et al.   HUMAN MOLECULAR GENETICS

Physiological functions and pathobiology of TDP-43 and FUS/TLS proteins

(2016) Antonia Ratti et al.   JOURNAL OF NEUROCHEMISTRY

Deficiency of the Survival of Motor Neuron Protein Impairs mRNA Localization and Local Translation in the Growth Cone of Motor Neurons

(2016) Claudia Fallini et al.   JOURNAL OF NEUROSCIENCE

Role of BMP receptor traffic in synaptic growth defects in an ALS model

(2016) Mugdha Deshpande et al.   MOLECULAR BIOLOGY OF THE CELL

Decoding ALS: from genes to mechanism

(2016) J. Paul Taylor et al.   NATURE

The inhibition of TDP-43 mitochondrial localization blocks its neuronal toxicity

(2016) Wenzhang Wang et al.   NATURE MEDICINE

RNA-sequencing of a mouse-model of spinal muscular atrophy reveals tissue-wide changes in splicing of U12-dependent introns

(2016) Thomas Koed Doktor et al.   NUCLEIC ACIDS RESEARCH

Identification of consensus binding sites clarifies FMRP binding determinants

(2016) Bart R. Anderson et al.   NUCLEIC ACIDS RESEARCH

Glutamate-induced RNA localization and translation in neurons

(2016) Young J. Yoon et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

SMN - A chaperone for nuclear RNP social occasions?

(2016) Amanda C. Raimer et al.   RNA Biology

Principles and Properties of Stress Granules

(2016) David S.W. Protter et al.   TRENDS IN CELL BIOLOGY

Super-Resolution Microscopy Reveals Presynaptic Localization of the ALS/FTD Related Protein FUS in Hippocampal Neurons

(2016) Michael Schoen et al.   Frontiers in Cellular Neuroscience

Distinct and shared functions of ALS-associated proteins TDP-43, FUS and TAF15 revealed by multisystem analyses

(2016) Katannya Kapeli et al.   Nature Communications

ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function

(2016) Aarti Sharma et al.   Nature Communications

TIA1 oxidation inhibits stress granule assembly and sensitizes cells to stress-induced apoptosis

(2016) Kyoko Arimoto-Matsuzaki et al.   Nature Communications

Enhanced CLIP Uncovers IMP Protein-RNA Targets in Human Pluripotent Stem Cells Important for Cell Adhesion and Survival

(2016) Anne E. Conway et al.   Cell Reports

Interaction of tau with the RNA-Binding Protein TIA1 Regulates tau Pathophysiology and Toxicity

(2016) Tara Vanderweyde et al.   Cell Reports

NOVA regulates Dcc alternative splicing during neuronal migration and axon guidance in the spinal cord

(2016) Janelle C Leggere et al.   eLife

Sam68 promotes self-renewal and glycolytic metabolism in mouse neural progenitor cells by modulating Aldh1a3 pre-mRNA 3'-end processing

(2016) Piergiorgio La Rosa et al.   eLife

Drosophila Nanos acts as a molecular clamp that modulates the RNA-binding and repression activities of Pumilio

(2016) Chase A Weidmann et al.   eLife

NOVA2-mediated RNA regulation is required for axonal pathfinding during development

(2016) Yuhki Saito et al.   eLife

Altered mRNA Splicing in SMN-Depleted Motor Neuron-Like Cells

(2016) Sara K. Custer et al.   PLoS One

Cortical synaptic and dendritic spine abnormalities in a presymptomatic TDP-43 model of amyotrophic lateral sclerosis

(2016) Matthew J. Fogarty et al.   Scientific Reports

Loss of preimplantation embryo resulting from a Pum1 gene trap mutation

(2015) Chenwang Zhang et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Pumilio1 Haploinsufficiency Leads to SCA1-like Neurodegeneration by Increasing Wild-Type Ataxin1 Levels

(2015) Vincenzo A. Gennarino et al.   CELL

IGF2BP2/IMP2-Deficient Mice Resist Obesity through Enhanced Translation of Ucp1 mRNA and Other mRNAs Encoding Mitochondrial Proteins

(2015) Ning Dai et al.   Cell Metabolism

KH–RNA interactions: back in the groove

(2015) Giuseppe Nicastro et al.   CURRENT OPINION IN STRUCTURAL BIOLOGY

Reduced Pumilio-2 expression in patients with temporal lobe epilepsy and in the lithium–pilocarpine induced epilepsy rat model

(2015) Xu-Ling Wu et al.   EPILEPSY & BEHAVIOR

Position-specific binding of FUS to nascent RNA regulates mRNA length

(2015) Akio Masuda et al.   GENES & DEVELOPMENT

Mammalian Target of Rapamycin (mTOR) Tagging Promotes Dendritic Branch Variability through the Capture of Ca2+/Calmodulin-dependent Protein Kinase II α (CaMKIIα) mRNAs by the RNA-binding Protein HuD

(2015) Natasha M. Sosanya et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

SAM68 is a physiological regulator ofSMN2splicing in spinal muscular atrophy

(2015) Vittoria Pagliarini et al.   JOURNAL OF CELL BIOLOGY

Specific interaction of KIF11 with ZBP1 regulates the transport of  -actin mRNA and cell motility

(2015) T. Song et al.   JOURNAL OF CELL SCIENCE

Overexpression of nuclear FUS induces neuronal cell death

(2015) H. Suzuki et al.   NEUROSCIENCE

FUS functions in coupling transcription to splicing by mediating an interaction between RNAP II and U1 snRNP

(2015) Yong Yu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

TDP-43 repression of nonconserved cryptic exons is compromised in ALS-FTD

(2015) J. P. Ling et al.   SCIENCE

The structural basis of Miranda-mediated Staufen localization during Drosophila neuroblast asymmetric division

(2015) Min Jia et al.   Nature Communications

ALS-causative mutations in FUS/TLS confer gain and loss of function by altered association with SMN and U1-snRNP

(2015) Shuying Sun et al.   Nature Communications

Fmrp Interacts with Adar and Regulates RNA Editing, Synaptic Density and Locomotor Activity in Zebrafish

(2015) Adi Shamay-Ramot et al.   PLoS Genetics

HuD Interacts with Bdnf mRNA and Is Essential for Activity-Induced BDNF Synthesis in Dendrites

(2015) Filip Vanevski et al.   PLoS One

Fmr1 and Nlgn3 knockout rats: Novel tools for investigating autism spectrum disorders.

(2014) Shannon M. Hamilton et al.   BEHAVIORAL NEUROSCIENCE

A Chromatin-Dependent Role of the Fragile X Mental Retardation Protein FMRP in the DNA Damage Response

(2014) Roman Alpatov et al.   CELL

mRNA-Binding Protein TIA-1 Reduces Cytokine Expression in Human Endometrial Stromal Cells and Is Down-Regulated in Ectopic Endometrium

(2014) Hakan Mete Karalok et al.   JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM

Futsch/MAP1B mRNA Is a Translational Target of TDP-43 and Is Neuroprotective in a Drosophila Model of Amyotrophic Lateral Sclerosis

(2014) A. N. Coyne et al.   JOURNAL OF NEUROSCIENCE

Prenatal Deletion of the RNA-Binding Protein HuD Disrupts Postnatal Cortical Circuit Maturation and Behavior

(2014) E. M. DeBoer et al.   JOURNAL OF NEUROSCIENCE

ALS-Linked Mutations Enlarge TDP-43-Enriched Neuronal RNA Granules in the Dendritic Arbor

(2014) L. Liu-Yesucevitz et al.   JOURNAL OF NEUROSCIENCE

FMRP regulates miR196a-mediated repression of HOXB8 via interaction with the AGO2 MID domain

(2014) Ying Li et al.   Molecular BioSystems

Fragile X Mental Retardation Protein Regulates Translation by Binding Directly to the Ribosome

(2014) Eileen Chen et al.   MOLECULAR CELL

MicroRNAs Trigger Dissociation of eIF4AI and eIF4AII from Target mRNAs in Humans

(2014) Akira Fukao et al.   MOLECULAR CELL

Chronological requirements of TDP-43 function in synaptic organization and locomotive control

(2014) Giulia Romano et al.   NEUROBIOLOGY OF DISEASE

Dysregulated expression of lipid storage and membrane dynamics factors in Tia1 knockout mouse nervous tissue

(2014) Melanie Vanessa Heck et al.   NEUROGENETICS

Axonal Transport of TDP-43 mRNA Granules Is Impaired by ALS-Causing Mutations

(2014) Nael H. Alami et al.   NEURON

Stress granules are dispensable for mRNA stabilization during cellular stress

(2014) Nadine Bley et al.   NUCLEIC ACIDS RESEARCH

Structure, dynamics and RNA binding of the multi-domain splicing factor TIA-1

(2014) Iren Wang et al.   NUCLEIC ACIDS RESEARCH

FMRP S499 Is Phosphorylated Independent of mTORC1-S6K1 Activity

(2014) Christopher M. Bartley et al.   PLoS One

Subcellular transcriptome alterations in a cell culture model of spinal muscular atrophy point to widespread defects in axonal growth and presynaptic differentiation

(2014) Lena Saal et al.   RNA

Single  -Actin mRNA Detection in Neurons Reveals a Mechanism for Regulating Its Translatability

(2014) A. R. Buxbaum et al.   SCIENCE

Promoter-Bound Trinucleotide Repeat mRNA Drives Epigenetic Silencing in Fragile X Syndrome

(2014) D. Colak et al.   SCIENCE

‘Black sheep’ that don’t leave the double-stranded RNA-binding domain fold

(2014) Michael L. Gleghorn et al.   TRENDS IN BIOCHEMICAL SCIENCES

Functionally diverse dendritic mRNAs rapidly associate with ribosomes following a novel experience

(2014) Joshua A. Ainsley et al.   Nature Communications

RNA binding proteins: a common denominator of neuronal function and dysfunction

(2014) Epaminondas Doxakis   Neuroscience Bulletin

HuD Regulates Coding and Noncoding RNA to Induce APP→Aβ Processing

(2014) Min-Ju Kang et al.   Cell Reports

Immunopathogenesis of paraneoplastic neurological syndromes associated with anti-Hu antibodies

(2014) Béatrice SL Pignolet et al.   OncoImmunology

Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALS

(2013) Tania F. Gendron et al.   ACTA NEUROPATHOLOGICA

Loss of TDP-43 causes age-dependent progressive motor neuron degeneration

(2013) Yohei Iguchi et al.   BRAIN

Motor neuron expression of the voltage-gated calcium channel cacophony restores locomotion defects in a Drosophila, TDP-43 loss of function model of ALS

(2013) Jer-Cherng Chang et al.   BRAIN RESEARCH

Altered Ribostasis: RNA-Protein Granules in Degenerative Disorders

(2013) Mani Ramaswami et al.   CELL

Dynamics of survival of motor neuron (SMN) protein interaction with the mRNA-binding protein IMP1 facilitates its trafficking into motor neuron axons

(2013) Claudia Fallini et al.   Developmental Neurobiology

Programmed Cell Death Is Impaired in the Developing Brain ofFmr1Mutants

(2013) Ying Cheng et al.   DEVELOPMENTAL NEUROSCIENCE

IMP2 regulates differentiation potentials of mouse neocortical neural precursor cells

(2013) Yuki Fujii et al.   GENES TO CELLS

ALS-associated mutations in FUS disrupt the axonal distribution and function of SMN

(2013) Ewout J.N. Groen et al.   HUMAN MOLECULAR GENETICS

ALS mutant FUS disrupts nuclear localization and sequesters wild-type FUS within cytoplasmic stress granules

(2013) Caroline Vance et al.   HUMAN MOLECULAR GENETICS

Degradation of high affinity HuD targets releases Kv1.1 mRNA from miR-129 repression by mTORC1

(2013) Natasha M. Sosanya et al.   JOURNAL OF CELL BIOLOGY

The RNA-binding protein Fus directs translation of localized mRNAs in APC-RNP granules

(2013) Kyota Yasuda et al.   JOURNAL OF CELL BIOLOGY

A HuD-ZBP1 ribonucleoprotein complex localizes GAP-43 mRNA into axons through its 3′ untranslated region AU-rich regulatory element

(2013) Soonmoon Yoo et al.   JOURNAL OF NEUROCHEMISTRY

Dendritic GluN2A Synthesis Mediates Activity-Induced NMDA Receptor Insertion

(2013) S. A. Swanger et al.   JOURNAL OF NEUROSCIENCE

CPEB1 coordinates alternative 3′-UTR formation with translational regulation

(2013) Felice-Alessio Bava et al.   NATURE

Interaction of FUS and HDAC1 regulates DNA damage response and repair in neurons

(2013) Wen-Yuan Wang et al.   NATURE NEUROSCIENCE

FMRP Regulates Neurotransmitter Release and Synaptic Information Transmission by Modulating Action Potential Duration via BK Channels

(2013) Pan-Yue Deng et al.   NEURON

All three RNA recognition motifs and the hinge region of HuC play distinct roles in the regulation of alternative splicing

(2013) Melissa N. Hinman et al.   NUCLEIC ACIDS RESEARCH

T-cell Intracellular Antigen (TIA)-Proteins Deficiency in Murine Embryonic Fibroblasts Alters Cell Cycle Progression and Induces Autophagy

(2013) Carmen Sánchez-Jiménez et al.   PLoS One

RNA-binding protein Sam68 controls synapse number and local  -actin mRNA metabolism in dendrites

(2013) M. E. Klein et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Distinct binding properties of TIAR RRMs and linker region

(2013) Henry S. Kim et al.   RNA Biology

Identification of In Vivo, Conserved, TAF15 RNA Binding Sites Reveals the Impact of TAF15 on the Neuronal Transcriptome

(2013) Fadia Ibrahim et al.   Cell Reports

Staufen2 Regulates Neuronal Target RNAs

(2013) Jacki E. Heraud-Farlow et al.   Cell Reports

A network of heterochronic genes including Imp1 regulates temporal changes in stem cell properties

(2013) Jinsuke Nishino et al.   eLife

NOVA-dependent regulation of cryptic NMD exons controls synaptic protein levels after seizure

(2013) Taesun Eom et al.   eLife

Localization of fused in sarcoma (FUS) protein to the post-synaptic density in the brain

(2012) Naoya Aoki et al.   ACTA NEUROPATHOLOGICA

An SMN-Dependent U12 Splicing Event Essential for Motor Circuit Function

(2012) Francesco Lotti et al.   CELL

Translational Control of Mitochondrial Energy Production Mediates Neuron Morphogenesis

(2012) Aparna Oruganty-Das et al.   Cell Metabolism

Asymmetric Segregation of the Double-Stranded RNA Binding Protein Staufen2 during Mammalian Neural Stem Cell Divisions Promotes Lineage Progression

(2012) Gretchen Kusek et al.   Cell Stem Cell

An Asymmetrically Localized Staufen2-Dependent RNA Complex Regulates Maintenance of Mammalian Neural Stem Cells

(2012) John P. Vessey et al.   Cell Stem Cell

Insulin-like growth factor 2 mRNA-binding proteins (IGF2BPs): post-transcriptional drivers of cancer progression?

(2012) Jessica L. Bell et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

RRM–RNA recognition: NMR or crystallography…and new findings

(2012) Gerrit M Daubner et al.   CURRENT OPINION IN STRUCTURAL BIOLOGY

FUS stimulates microRNA biogenesis by facilitating co-transcriptional Drosha recruitment

(2012) Mariangela Morlando et al.   EMBO JOURNAL

Spliceosome integrity is defective in the motor neuron diseases ALS and SMA

(2012) Hitomi Tsuiji et al.   EMBO Molecular Medicine

Domains involved in TAF15 subcellular localisation: Dependence on cell type and ongoing transcription

(2012) Marija Marko et al.   GENE

The ALS disease protein TDP-43 is actively transported in motor neuron axons and regulates axon outgrowth

(2012) Claudia Fallini et al.   HUMAN MOLECULAR GENETICS

TDP-43 and FUS RNA-binding Proteins Bind Distinct Sets of Cytoplasmic Messenger RNAs and Differently Regulate Their Post-transcriptional Fate in Motoneuron-like Cells

(2012) Claudia Colombrita et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

RNA Binding Proteins Accumulate at the Postsynaptic Density with Synaptic Activity

(2012) G. Zhang et al.   JOURNAL OF NEUROSCIENCE

Bidirectional Control of mRNA Translation and Synaptic Plasticity by the Cytoplasmic Polyadenylation Complex

(2012) Tsuyoshi Udagawa et al.   MOLECULAR CELL

FMRP targets distinct mRNA sequence elements to regulate protein expression

(2012) Manuel Ascano et al.   NATURE

Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs

(2012) Clotilde Lagier-Tourenne et al.   NATURE NEUROSCIENCE

Endogenous TDP-43 localized to stress granules can subsequently form protein aggregates

(2012) Sarah J. Parker et al.   NEUROCHEMISTRY INTERNATIONAL

Neuronal Elav-like (Hu) Proteins Regulate RNA Splicing and Abundance to Control Glutamate Levels and Neuronal Excitability

(2012) Gulayse Ince-Dunn et al.   NEURON

The Local Transcriptome in the Synaptic Neuropil Revealed by Deep Sequencing and High-Resolution Imaging

(2012) Iván J. Cajigas et al.   NEURON

HuR protein attenuates miRNA-mediated repression by promoting miRISC dissociation from the target RNA

(2012) Pradipta Kundu et al.   NUCLEIC ACIDS RESEARCH

Gene Expression Responses to FUS, EWS, and TAF15 Reduction and Stress Granule Sequestration Analyses Identifies FET-Protein Non-Redundant Functions

(2012) Jenny Blechingberg et al.   PLoS One

Staufen2 functions in Staufen1-mediated mRNA decay by binding to itself and its paralog and promoting UPF1 helicase but not ATPase activity

(2012) E. Park et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Neuronal mRNAs travel singly into dendrites

(2012) M. Batish et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

TLS/FUS (translocated in liposarcoma/fused in sarcoma) regulates target gene transcription via single-stranded DNA response elements

(2012) A. Y. Tan et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Position-dependent FUS-RNA interactions regulate alternative splicing events and transcriptions

(2012) Shinsuke Ishigaki et al.   Scientific Reports

Widespread binding of FUS along nascent RNA regulates alternative splicing in the brain

(2012) Boris Rogelj et al.   Scientific Reports

Identification of RNA bound to the TDP-43 ribonucleoprotein complex in the adult mouse brain

(2012) Ramesh K. Narayanan et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Mutational analysis reveals the FUS homolog TAF15 as a candidate gene for familial amyotrophic lateral sclerosis

(2011) N. Ticozzi et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART B-NEUROPSYCHIATRIC GENETICS

Microtubule association of a neuronal RNA-binding protein HuD through its binding to the light chain of MAP1B

(2011) Yuki Fujiwara et al.   BIOCHIMIE

Genome wide identification of Staufen2-bound mRNAs in embryonic rat brains

(2011) Marjolaine Maher-Laporte et al.   BMB Reports

Pathological hallmarks of amyotrophic lateral sclerosis/frontotemporal lobar degeneration in transgenic mice produced with TDP-43 genomic fragments

(2011) Vivek Swarup et al.   BRAIN

FET proteins TAF15 and EWS are selective markers that distinguish FTLD with FUS pathology from amyotrophic lateral sclerosis with FUS mutations

(2011) Manuela Neumann et al.   BRAIN

Neuralized1 Activates CPEB3: A Function for Nonproteolytic Ubiquitin in Synaptic Plasticity and Memory Storage

(2011) Elias Pavlopoulos et al.   CELL

FMRP Stalls Ribosomal Translocation on mRNAs Linked to Synaptic Function and Autism

(2011) Jennifer C. Darnell et al.   CELL

SAM68 Regulates Neuronal Activity-Dependent Alternative Splicing of Neurexin-1

(2011) Takatoshi Iijima et al.   CELL

SMN Deficiency Reduces Cellular Ability to Form Stress Granules, Sensitizing Cells to Stress

(2011) Tie Zou et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

Fragile X mental retardation protein is required for programmed cell death and clearance of developmentally-transient peptidergic neurons

(2011) Cheryl L. Gatto et al.   DEVELOPMENTAL BIOLOGY

Limited availability of ZBP1 restricts axonal mRNA localization and nerve regeneration capacity

(2011) Christopher J Donnelly et al.   EMBO JOURNAL

Pan-ethnic carrier screening and prenatal diagnosis for spinal muscular atrophy: clinical laboratory analysis of >72 400 specimens

(2011) Elaine A Sugarman et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

Deregulation of the A-to-I RNA editing mechanism in psychiatric disorders

(2011) Gilad Silberberg et al.   HUMAN MOLECULAR GENETICS

In vivo neuronal function of the fragile X mental retardation protein is regulated by phosphorylation

(2011) R. L. Coffee et al.   HUMAN MOLECULAR GENETICS

The spinal muscular atrophy disease protein SMN is linked to the rho-kinase pathway via profilin

(2011) Anna Nölle et al.   HUMAN MOLECULAR GENETICS

TAR DNA-binding protein 43 (TDP-43) regulates stress granule dynamics via differential regulation of G3BP and TIA-1

(2011) Karli K. McDonald et al.   HUMAN MOLECULAR GENETICS

The ALS-associated proteins FUS and TDP-43 function together to affect Drosophila locomotion and life span

(2011) Ji-Wu Wang et al.   JOURNAL OF CLINICAL INVESTIGATION

Characterization of a Family of RanBP2-Type Zinc Fingers that Can Recognize Single-Stranded RNA

(2011) Cuong D. Nguyen et al.   JOURNAL OF MOLECULAR BIOLOGY

UG Repeats/TDP-43 Interactions near 5′ Splice Sites Exert Unpredictable Effects on Splicing Modulation

(2011) Monica Passoni et al.   JOURNAL OF MOLECULAR BIOLOGY

The Survival of Motor Neuron (SMN) Protein Interacts with the mRNA-Binding Protein HuD and Regulates Localization of Poly(A) mRNA in Primary Motor Neuron Axons

(2011) C. Fallini et al.   JOURNAL OF NEUROSCIENCE

Zipcode Binding Protein 1 Regulates the Development of Dendritic Arbors in Hippocampal Neurons

(2011) M. Perycz et al.   JOURNAL OF NEUROSCIENCE

Netrin-1-Induced Local  -Actin Synthesis and Growth Cone Guidance Requires Zipcode Binding Protein 1

(2011) K. Welshhans et al.   JOURNAL OF NEUROSCIENCE

Staufen 2 regulates mGluR long-term depression and Map1b mRNA distribution in hippocampal neurons

(2011) G. Lebeau et al.   LEARNING & MEMORY

RNA targets of TDP-43 identified by UV-CLIP are deregulated in ALS

(2011) Shangxi Xiao et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Reversible Inhibition of PSD-95 mRNA Translation by miR-125a, FMRP Phosphorylation, and mGluR Signaling

(2011) Ravi S. Muddashetty et al.   MOLECULAR CELL

Sam68 Is Required for Both NF-κB Activation and Apoptosis Signaling by the TNF Receptor

(2011) Parameswaran Ramakrishnan et al.   MOLECULAR CELL

Integrative Regulatory Mapping Indicates that the RNA-Binding Protein HuR Couples Pre-mRNA Processing and mRNA Stability

(2011) Neelanjan Mukherjee et al.   MOLECULAR CELL

Transcriptome-wide Analysis of Regulatory Interactions of the RNA-Binding Protein HuR

(2011) Svetlana Lebedeva et al.   MOLECULAR CELL

Activity Regulation of Adenosine Deaminases Acting on RNA (ADARs)

(2011) Cesare Orlandi et al.   MOLECULAR NEUROBIOLOGY

Peripheral SMN restoration is essential for long-term rescue of a severe spinal muscular atrophy mouse model

(2011) Yimin Hua et al.   NATURE

Modulation of dADAR-dependent RNA editing by the Drosophila fragile X mental retardation protein

(2011) Balpreet Bhogal et al.   NATURE NEUROSCIENCE

Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43

(2011) Magdalini Polymenidou et al.   NATURE NEUROSCIENCE

Characterizing the RNA targets and position-dependent splicing regulation by TDP-43

(2011) James R Tollervey et al.   NATURE NEUROSCIENCE

Total RNA sequencing reveals nascent transcription and widespread co-transcriptional splicing in the human brain

(2011) Adam Ameur et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

A new model to study spinal muscular atrophy: Neurite degeneration and cell death is counteracted by BCL-XL Overexpression in motoneurons

(2011) Ana Garcera et al.   NEUROBIOLOGY OF DISEASE

TDP-43 regulates global translational yield by splicing of exon junction complex component SKAR

(2011) Fabienne C. Fiesel et al.   NUCLEIC ACIDS RESEARCH

TDP-43 Regulates Drosophila Neuromuscular Junctions Growth by Modulating Futsch/MAP1B Levels and Synaptic Microtubules Organization

(2011) Vinay K. Godena et al.   PLoS One

Genome-Wide Association Study of Schizophrenia in Japanese Population

(2011) Kazuo Yamada et al.   PLoS One

Pumilio-2 Function in the Mouse Nervous System

(2011) Henrike Siemen et al.   PLoS One

Interaction of survival of motor neuron (SMN) and HuD proteins with mRNA cpg15 rescues motor neuron axonal deficits

(2011) B. Akten et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Hu proteins regulate alternative splicing by inducing localized histone hyperacetylation in an RNA-dependent manner

(2011) H.-L. Zhou et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A yeast functional screen predicts new candidate ALS disease genes

(2011) J. Couthouis et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

FUS and TARDBP but Not SOD1 Interact in Genetic Models of Amyotrophic Lateral Sclerosis

(2011) Edor Kabashi et al.   PLoS Genetics

FUS Transgenic Rats Develop the Phenotypes of Amyotrophic Lateral Sclerosis and Frontotemporal Lobar Degeneration

(2011) Cao Huang et al.   PLoS Genetics

Sam68 sequestration and partial loss of function are associated with splicing alterations in FXTAS patients

(2010) Chantal Sellier et al.   EMBO JOURNAL

The splicing regulator Sam68 binds to a novel exonic splicing silencer and functions in SMN2 alternative splicing in spinal muscular atrophy

(2010) Simona Pedrotti et al.   EMBO JOURNAL

Antisense correction of SMN2 splicing in the CNS rescues necrosis in a type III SMA mouse model

(2010) Y. Hua et al.   GENES & DEVELOPMENT

Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granules

(2010) Daryl A. Bosco et al.   HUMAN MOLECULAR GENETICS

The heterogeneous nuclear ribonucleoprotein-R is necessary for axonal β-actin mRNA translocation in spinal motor neurons

(2010) Michael Glinka et al.   HUMAN MOLECULAR GENETICS

Identification of Neuronal RNA Targets of TDP-43-containing Ribonucleoprotein Complexes

(2010) Chantelle F. Sephton et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Elevated expression of TDP-43 in the forebrain of mice is sufficient to cause neurological and pathological phenotypes mimicking FTLD-U

(2010) Kuen-Jer Tsai et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Fragile X Mental Retardation Protein Is Required for Rapid Experience-Dependent Regulation of the Potassium Channel Kv3.1b

(2010) J. G. Strumbos et al.   JOURNAL OF NEUROSCIENCE

Delayed Stabilization of Dendritic Spines in Fragile X Mice

(2010) A. Cruz-Martin et al.   JOURNAL OF NEUROSCIENCE

miR-375 Inhibits Differentiation of Neurites by Lowering HuD Levels

(2010) K. Abdelmohsen et al.   MOLECULAR AND CELLULAR BIOLOGY

TDP-43 Is Directed to Stress Granules by Sorbitol, a Novel Physiological Osmotic and Oxidative Stressor

(2010) C. M. Dewey et al.   MOLECULAR AND CELLULAR BIOLOGY

TAR DNA-binding protein 43 in neurodegenerative disease

(2010) Alice S. Chen-Plotkin et al.   Nature Reviews Neurology

Genetic contribution of FUS to frontotemporal lobar degeneration

(2010) T. Van Langenhove et al.   NEUROLOGY

Nova2 Regulates Neuronal Migration through an RNA Switch in Disabled-1 Signaling

(2010) Masato Yano et al.   NEURON

Regulation of Synaptic Structure and Function by FMRP-Associated MicroRNAs miR-125b and miR-132

(2010) Dieter Edbauer et al.   NEURON

Splicing factor and exon profiling across human tissues

(2010) Pierre de la Grange et al.   NUCLEIC ACIDS RESEARCH

Impaired Embryonic Development in Mice Overexpressing the RNA-Binding Protein TIAR

(2010) Yacine Kharraz et al.   PLoS One

Tar DNA Binding Protein-43 (TDP-43) Associates with Stress Granules: Analysis of Cultured Cells and Pathological Brain Tissue

(2010) Liqun Liu-Yesucevitz et al.   PLoS One

Mammalian Pumilio 2 regulates dendrite morphogenesis and synaptic function

(2010) J. P. Vessey et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The neuronal splicing factor Nova co-localizes with target RNAs in the dendrite

(2010) Racca   Frontiers in Neural Circuits

iCLIP Predicts the Dual Splicing Effects of TIA-RNA Interactions

(2010) Zhen Wang et al.   PLOS BIOLOGY

HITS-CLIP: panoramic views of protein-RNA regulation in living cells

(2010) Robert B. Darnell   Wiley Interdisciplinary Reviews-RNA

PAR-CliP - A Method to Identify Transcriptome-wide the Binding Sites of RNA Binding Proteins

(2010) Markus Hafner et al.   Jove-Journal of Visualized Experiments

A new subtype of frontotemporal lobar degeneration with FUS pathology

(2009) M. Neumann et al.   BRAIN

Sam68 relocalization into stress granules in response to oxidative stress through complexing with TIA-1

(2009) Jorge Henao-Mejia et al.   EXPERIMENTAL CELL RESEARCH

Making synaptic plasticity and memory last: mechanisms of translational regulation

(2009) J. D. Richter et al.   GENES & DEVELOPMENT

Sam68 regulates translation of target mRNAs in male germ cells, necessary for mouse spermatogenesis

(2009) Maria Paola Paronetto et al.   JOURNAL OF CELL BIOLOGY

Mammalian Staufen 1 is recruited to stress granules and impairs their assembly

(2009) M. G. Thomas et al.   JOURNAL OF CELL SCIENCE

Equal numbers of neuronal and nonneuronal cells make the human brain an isometrically scaled-up primate brain

(2009) Frederico A.C. Azevedo et al.   JOURNAL OF COMPARATIVE NEUROLOGY

TDP-43 is recruited to stress granules in conditions of oxidative insult

(2009) Claudia Colombrita et al.   JOURNAL OF NEUROCHEMISTRY

TDP-43 localizes in mRNA transcription and processing sites in mammalian neurons

(2009) Iñigo Casafont et al.   JOURNAL OF STRUCTURAL BIOLOGY

The ELAV Protein HuD Stimulates Cap-Dependent Translation in a Poly(A)- and eIF4A-Dependent Manner

(2009) Akira Fukao et al.   MOLECULAR CELL

RNA processing and its regulation: global insights into biological networks

(2009) Donny D. Licatalosi et al.   NATURE REVIEWS GENETICS

Rescuing Z+ agrin splicing in Nova null mice restores synapse formation and unmasks a physiologic defect in motor neuron firing

(2009) M. Ruggiu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Pumilio 2 controls translation by competing with eIF4E for 7-methyl guanosine cap recognition

(2009) Q. Cao et al.   RNA

Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6

(2009) C. Vance et al.   SCIENCE

Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis

(2009) T. J. Kwiatkowski et al.   SCIENCE

Frontotemporal dementia and amyotrophic lateral sclerosis-associated disease protein TDP-43 promotes dendritic branching

(2009) Yubing Lu et al.   Molecular Brain

A Mouse Model of the Human Fragile X Syndrome I304N Mutation

(2009) Julie B. Zang et al.   PLoS Genetics

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

(2008) Mattias K Andersson et al.   BMC CELL BIOLOGY

The Fragile X Syndrome Protein Represses Activity-Dependent Translation through CYFIP1, a New 4E-BP

(2008) Ilaria Napoli et al.   CELL

A Direct Role for FMRP in Activity-Dependent Dendritic mRNA Transport Links Filopodial-Spine Morphogenesis to Fragile X Syndrome

(2008) Jason B. Dictenberg et al.   DEVELOPMENTAL CELL

PRMT1 mediated methylation of TAF15 is required for its positive gene regulatory function

(2008) Laure Jobert et al.   EXPERIMENTAL CELL RESEARCH

A systematic analysis of intronic sequences downstream of 5' splice sites reveals a widespread role for U-rich motifs and TIA1/TIAL1 proteins in alternative splicing regulation

(2008) I. Aznarez et al.   GENOME RESEARCH

Replication of association between ELAVL4 and Parkinson disease: the GenePD study

(2008) Anita L. DeStefano et al.   HUMAN GENETICS

Reduced SMN protein impairs maturation of the neuromuscular junctions in mouse models of spinal muscular atrophy

(2008) Shingo Kariya et al.   HUMAN MOLECULAR GENETICS

Post-transcriptional Regulation of Neuro-oncological Ventral Antigen 1 by the Neuronal RNA-binding Proteins ELAV

(2008) Antonia Ratti et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Structural determinants of the cellular localization and shuttling of TDP-43

(2008) Y. M. Ayala et al.   JOURNAL OF CELL SCIENCE

Activity-dependent expression of ELAV/Hu RBPs and neuronal mRNAs in seizure and cocaine brain

(2008) Dhanrajan M. Tiruchinapalli et al.   JOURNAL OF NEUROCHEMISTRY

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

(2008) I.-Fan Wang et al.   JOURNAL OF NEUROCHEMISTRY

Pumilio Binds para mRNA and Requires Nanos and Brat to Regulate Sodium Current in Drosophila Motoneurons

(2008) N. I. Muraro et al.   JOURNAL OF NEUROSCIENCE

A Molecular Circuit Composed of CPEB-1 and c-Jun Controls Growth Hormone-Mediated Synaptic Plasticity in the Mouse Hippocampus

(2008) N. R. Zearfoss et al.   JOURNAL OF NEUROSCIENCE

Staufen1 Regulation of Protein Synthesis-Dependent Long-Term Potentiation and Synaptic Function in Hippocampal Pyramidal Cells

(2008) G. Lebeau et al.   MOLECULAR AND CELLULAR BIOLOGY

HITS-CLIP yields genome-wide insights into brain alternative RNA processing

(2008) Donny D. Licatalosi et al.   NATURE

Induced ncRNAs allosterically modify RNA-binding proteins in cis to inhibit transcription

(2008) Xiangting Wang et al.   NATURE

Expression of 24,426 human alternative splicing events and predicted cis regulation in 48 tissues and cell lines

(2008) John C Castle et al.   NATURE GENETICS

Deep surveying of alternative splicing complexity in the human transcriptome by high-throughput sequencing

(2008) Qun Pan et al.   NATURE GENETICS

Motility Screen Identifies Drosophila IGF-II mRNA-Binding Protein—Zipcode-Binding Protein Acting in Oogenesis and Synaptogenesis

(2008) Kristin L. M. Boylan et al.   PLoS Genetics

The RNA binding protein CPEB regulates dendrite morphogenesis and neuronal circuit assembly in vivo

(2008) J. E. Bestman et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A loss of function allele for murine Staufen1 leads to impairment of dendritic Staufen1-RNP delivery and dendritic spine morphogenesis

(2008) J. P. Vessey et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Control of c-myc mRNA stability by IGF2BP1-associated cytoplasmic RNPs

(2008) D. Weidensdorfer et al.   RNA

RNA–protein interactions and control of mRNA stability in neurons

(2007) Federico Bolognani et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Dynamic Association of the Fragile X Mental Retardation Protein as a Messenger Ribonucleoprotein between Microtubules and Polyribosomes

(2007) Houping Wang et al.   MOLECULAR BIOLOGY OF THE CELL