Nuclear depletion of RNA-binding protein ELAVL3 (HuC) in sporadic and familial amyotrophic lateral sclerosis

Nucleolar stress in C9orf72 and sporadic ALS spinal motor neurons precedes TDP-43 mislocalization

(2021) Olubankole Aladesuyi Arogundade et al.   Acta Neuropathologica Communications

Cross-Comparison of Human iPSC Motor Neuron Models of Familial and Sporadic ALS Reveals Early and Convergent Transcriptomic Disease Signatures

(2020) Ritchie Ho et al.   Cell Systems

ALS-implicated protein TDP-43 sustains levels of STMN2, a mediator of motor neuron growth and repair

(2019) Joseph R. Klim et al.   NATURE NEUROSCIENCE

Premature polyadenylation-mediated loss of stathmin-2 is a hallmark of TDP-43-dependent neurodegeneration

(2019) Ze’ev Melamed et al.   NATURE NEUROSCIENCE

ALS-linked FUS mutations confer loss and gain of function in the nucleus by promoting excessive formation of dysfunctional paraspeckles

(2019) Haiyan An et al.   Acta Neuropathologica Communications

Purification of cross-linked RNA-protein complexes by phenol-toluol extraction

(2019) Erika C. Urdaneta et al.   Nature Communications

Cytoplasmic TDP-43 De-mixing Independent of Stress Granules Drives Inhibition of Nuclear Import, Loss of Nuclear TDP-43, and Cell Death

(2019) Fatima Gasset-Rosa et al.   NEURON

The debated toxic role of aggregated TDP-43 in amyotrophic lateral sclerosis: a resolution in sight?

(2019) Rudolf C Hergesheimer et al.   BRAIN

Update on amyotrophic lateral sclerosis genetics

(2019) David Brenner et al.   CURRENT OPINION IN NEUROLOGY

Structural basis for reversible amyloids of hnRNPA1 elucidates their role in stress granule assembly

(2019) Xinrui Gui et al.   Nature Communications

Elav-Mediated Exon Skipping and Alternative Polyadenylation of the Dscam1 Gene Are Required for Axon Outgrowth

(2019) Zhiping Zhang et al.   Cell Reports

Mutant FUS and ELAVL4 (HuD) Aberrant Crosstalk in Amyotrophic Lateral Sclerosis

(2019) Riccardo De Santis et al.   Cell Reports

ALS Genetics, Mechanisms, and Therapeutics: Where Are We Now?

(2019) Rita Mejzini et al.   Frontiers in Neuroscience

Transcriptome–pathology correlation identifies interplay between TDP-43 and the expression of its kinase CK1E in sporadic ALS

(2018) Florian Krach et al.   ACTA NEUROPATHOLOGICA

The AU-rich element landscape across human transcriptome reveals a large proportion in introns and regulation by ELAVL1/HuR

(2018) Tala Bakheet et al.   Biochimica et Biophysica Acta-Gene Regulatory Mechanisms

Motor neuron disease of paraneoplastic origin: a rare but treatable condition

(2018) Nicolas Mélé et al.   JOURNAL OF NEUROLOGY

Genome-wide Analyses Identify KIF5A as a Novel ALS Gene

(2018) Aude Nicolas et al.   NEURON

Elavl3 regulates neuronal polarity through the alternative splicing of an embryo-specific exon in AnkyrinG

(2018) Yuki Ogawa et al.   NEUROSCIENCE RESEARCH

Elavl3 is essential for the maintenance of Purkinje neuron axons

(2018) Yuki Ogawa et al.   Scientific Reports

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

(2017) Katannya Kapeli et al.   HUMAN GENETICS

Genetic and Pathological Assessment of hnRNPA1, hnRNPA2/B1, and hnRNPA3 in Familial and Sporadic Amyotrophic Lateral Sclerosis

(2017) Jennifer A. Fifita et al.   Neurodegenerative Diseases

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

ALS Along the Axons – Expression of Coding and Noncoding RNA Differs in Axons of ALS models

(2017) Nimrod Rotem et al.   Scientific Reports

Decoding ALS: from genes to mechanism

(2016) J. Paul Taylor et al.   NATURE

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

(2016) Katannya Kapeli et al.   Nature Communications

Regulatory consequences of neuronal ELAV-like protein binding to coding and non-coding RNAs in human brain

(2016) Claudia Scheckel et al.   eLife

Pathogenesis of FUS-associated ALS and FTD: insights from rodent models

(2016) Matthew Nolan et al.   Acta Neuropathologica Communications

From transcriptomic to protein level changes in TDP-43 and FUS loss-of-function cell models

(2015) Claudia Colombrita et al.   Biochimica et Biophysica Acta-Gene Regulatory Mechanisms

ALS-linked mutations in ubiquilin-2 or hnRNPA1 reduce interaction between ubiquilin-2 and hnRNPA1

(2015) Kathleen M. Gilpin et al.   HUMAN MOLECULAR GENETICS

AREsite2: an enhanced database for the comprehensive investigation of AU/GU/U-rich elements

(2015) Jörg Fallmann et al.   NUCLEIC ACIDS RESEARCH

Alternative polyadenylation coupled to transcription initiation: Insights from ELAV-mediated 3′ UTR extension

(2015) Valérie Hilgers   RNA Biology

Post-Transcriptional Up-Regulation of PDGF-C by HuR in Advanced and Stressed Breast Cancer

(2014) Nian-An Luo et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

HuR and TIA1/TIAL1 Are Involved in Regulation of Alternative Splicing of SIRT1 Pre-mRNA

(2014) Wenhui Zhao et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis

(2014) Janel O Johnson et al.   NATURE NEUROSCIENCE

Aggregate formation prevents dTDP-43 neurotoxicity in the Drosophila melanogaster eye

(2014) Lucia Cragnaz et al.   NEUROBIOLOGY OF DISEASE

Protease 2A induces stress granule formation during coxsackievirus B3 and enterovirus 71 infections

(2014) Shuo Wu et al.   Virology Journal

Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS

(2013) Hong Joo Kim et al.   NATURE

Posttranscriptional regulation of SOD1 gene expression under oxidative stress: Potential role of ELAV proteins in sporadic ALS

(2013) Pamela Milani et al.   NEUROBIOLOGY OF DISEASE

TDP-43-mediated neurodegeneration: towards a loss-of-function hypothesis?

(2013) Lies Vanden Broeck et al.   TRENDS IN MOLECULAR MEDICINE

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

(2012) Gulayse Ince-Dunn et al.   NEURON

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

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

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

Amyotrophic lateral sclerosis

(2011) Matthew C Kiernan et al.   LANCET

The maintenance of Purkinje axons by an RNA-binding protein HuC in adult cerebellum

(2011) Kyoko Kakumoto et al.   NEUROSCIENCE RESEARCH

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

Cytoplasmic Mislocalization of TDP-43 Is Toxic to Neurons and Enhanced by a Mutation Associated with Familial Amyotrophic Lateral Sclerosis

(2010) S. J. Barmada et al.   JOURNAL OF NEUROSCIENCE

The maintenance of Purkinje axons by HuC in adult cerebellum

(2010) Kyoko Kakumoto et al.   NEUROSCIENCE RESEARCH

Sporadic ALS has compartment-specific aberrant exon splicing and altered cell–matrix adhesion biology

(2009) Stuart J. Rabin et al.   HUMAN MOLECULAR GENETICS

Amyotrophic lateral sclerosis

(2009) Lokesh C Wijesekera et al.   Orphanet Journal of Rare Diseases

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

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

Diverse molecular functions of Hu proteins

(2008) M. N. Hinman et al.   CELLULAR AND MOLECULAR LIFE SCIENCES