ArticleNeuron2016
Protein-RNA Networks Regulated by Normal and ALS-Associated Mutant HNRNPA2B1 in the Nervous System.
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In one paragraph
Article in Neuron, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 119 papers.
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119citing papers in PubMed
14.6field-weighted citation impact, top 1% of its field
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Who cites it
119 citing papers in PubMed, 188 citations in OpenAlex.
- Mechanistic studies on HNRNPA2B1 suggest binding but not selective recognition of mRNA biology · 2026Article
- RNA G-quadruplex-protein interactions: from nuclear RNA processing to cytoplasmic stress response and neurodegeneration.RNA biology · 2026Review
- Neurodegenerative-related gene rescue in anterior insula after early life maltreatment restores adult social interaction in male mice.Molecular psychiatry · 2026Article
- HnRNP A1 and A2B1 enforce Ezh2 mRNA splicing to promote germinal center B cell responses.Nature communications · 2026Article
- Upregulated PKM2 Protects Dopaminergic Neurons From Oxidative Damage Through Nrf2 Transactivation in an MPTP-Induced Mouse Model of Parkinson's Disease.CNS neuroscience & therapeutics · 2026Article
- Full-Length Transcriptome Profiles Reveal the Molecular Function of Oncogene HNRNPA2B1 in Triple-Negative Breast Cancer.Cancer medicine · 2026Article
- Widespread hnRNP K Mislocalisation Suggests Differential Neuronal Vulnerability in the Neurodegenerative and Ageing Human Brain.Neuropathology and applied neurobiology · 2026Article
- tRNA synthetase activity is required for stress granule and P-body assembly.Genes & development · 2026Article
- Inverted Alu repeats in loop-out exon skipping across hominoid evolution.Nucleic acids research · 2026Article
- A role for the cholinergic neuron circadian clock in RNA metabolism and mediating neurodegeneration.Life science alliance · 2026Article
- TDP-43 directly inhibits mRNA accumulation in neurites through modulation of mRNA stability.The EMBO journal · 2026Article
- Transcriptomic and pathological analysis of the hnRNP network reveals glial involvement in frontotemporal lobar degeneration pathological subtypes.Brain communications · 2026Article
- The RNA-binding protein HNRNPA2B1 regulates neurite RNA abundance and motor-dependent cargo transport.Molecular biology of the cell · 2025Article
- Nanobodies targeting hnRNPA2/B1 and tau.bioRxiv : the preprint server for biology · 2025Article
- Integrated profiling of iPSC-derived motor neurons carrying C9orf72, FUS, TARDBP, or SOD1 mutations.Stem cell reports · 2025Article
- Spliceosome protein alterations differentiate hubs of the default mode connectome during the progression of Alzheimer's disease.Brain pathology (Zurich, Switzerland) · 2025Article
- U1-70K and U1A and tau pathogenesis in demented and non-demented individuals with Down syndrome.Alzheimer's & dementia : the journal of the Alzheimer's Association · 2025Article
- M6A-dependent RNA condensation underlies FUS autoregulation and can be harnessed for ALS therapy development.Science advances · 2025Article
- Evaluation of novel computational methods to identify RNA-binding protein footprints from structural data.RNA (New York, N.Y.) · 2025Article
- Stathmin-2 enhances motor axon regeneration after injury independent of its binding to tubulin.Proceedings of the National Academy of Sciences of the United States of America · 2025Article
59 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
27 authors at 8 institutions in 2 countries.
Fernando J MartinezDepartment of Cellular and Molecular Medicine, University of California, San Diego, La Jolla, CA 92093, USA; Stem Cell Program and Institute for Genomic Medicine, University of California, San Diego, La Jolla, CA 92093, USA.
Gabriel A PrattDepartment of Cellular and Molecular Medicine, University of California, San Diego, La Jolla, CA 92093, USA; Stem Cell Program and Institute for Genomic Medicine, University of California, San Diego, La Jolla, CA 92093, USA; Department of Bioinformatics and Systems Biology, University of California, San Diego, La Jolla, CA 92093, USA.
Eric L Van NostrandDepartment of Cellular and Molecular Medicine, University of California, San Diego, La Jolla, CA 92093, USA; Stem Cell Program and Institute for Genomic Medicine, University of California, San Diego, La Jolla, CA 92093, USA.
Ranjan BatraDepartment of Cellular and Molecular Medicine, University of California, San Diego, La Jolla, CA 92093, USA; Stem Cell Program and Institute for Genomic Medicine, University of California, San Diego, La Jolla, CA 92093, USA.
Stephanie C HuelgaDepartment of Cellular and Molecular Medicine, University of California, San Diego, La Jolla, CA 92093, USA; Stem Cell Program and Institute for Genomic Medicine, University of California, San Diego, La Jolla, CA 92093, USA.
Katannya KapeliDepartment of Cellular and Molecular Medicine, University of California, San Diego, La Jolla, CA 92093, USA; Stem Cell Program and Institute for Genomic Medicine, University of California, San Diego, La Jolla, CA 92093, USA; Department of Physiology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore 117597, Singapore.
Peter FreeseDepartment of Biology, MIT, Cambridge, MA 02139, USA.
Seung J ChunIonis Pharmaceuticals, Carlsbad, CA 92010, USA.
Karen LingIonis Pharmaceuticals, Carlsbad, CA 92010, USA.
Chelsea Gelboin-BurkhartDepartment of Cellular and Molecular Medicine, University of California, San Diego, La Jolla, CA 92093, USA; Stem Cell Program and Institute for Genomic Medicine, University of California, San Diego, La Jolla, CA 92093, USA.
Layla FijanyDepartment of Cellular and Molecular Medicine, University of California, San Diego, La Jolla, CA 92093, USA; Stem Cell Program and Institute for Genomic Medicine, University of California, San Diego, La Jolla, CA 92093, USA.
Harrison C WangDepartment of Cellular and Molecular Medicine, University of California, San Diego, La Jolla, CA 92093, USA; Stem Cell Program and Institute for Genomic Medicine, University of California, San Diego, La Jolla, CA 92093, USA.
Julia K NussbacherDepartment of Cellular and Molecular Medicine, University of California, San Diego, La Jolla, CA 92093, USA; Stem Cell Program and Institute for Genomic Medicine, University of California, San Diego, La Jolla, CA 92093, USA.
Sara M BroskiTaube/Koret Center for Neurodegenerative Disease Research, Gladstone Institute of Neurological Disease, San Francisco, CA 94158, USA.
Hong Joo KimHoward Hughes Medical Institute, Department of Cell and Molecular Biology, St. Jude Children's Research Hospital, Memphis, TN 38105, USA.
Rea LardelliDivision of Biological Sciences, University of California, San Diego, La Jolla, CA 92093, USA.
Balaji SundararamanDepartment of Cellular and Molecular Medicine, University of California, San Diego, La Jolla, CA 92093, USA; Stem Cell Program and Institute for Genomic Medicine, University of California, San Diego, La Jolla, CA 92093, USA.
John P DonohueDepartment of Molecular, Cell, and Developmental Biology, Sinsheimer Labs, University of California, Santa Cruz, Santa Cruz, CA 95064, USA.
Ashkan JavaherianTaube/Koret Center for Neurodegenerative Disease Research, Gladstone Institute of Neurological Disease, San Francisco, CA 94158, USA.
Jens Lykke-AndersenDivision of Biological Sciences, University of California, San Diego, La Jolla, CA 92093, USA.
Steven FinkbeinerTaube/Koret Center for Neurodegenerative Disease Research, Gladstone Institute of Neurological Disease, San Francisco, CA 94158, USA; Departments of Neurology and Physiology, University of California, San Francisco, San Francisco, CA 94107, USA.
C Frank BennettIonis Pharmaceuticals, Carlsbad, CA 92010, USA.
Manuel AresDepartment of Molecular, Cell, and Developmental Biology, Sinsheimer Labs, University of California, Santa Cruz, Santa Cruz, CA 95064, USA.
Christopher B BurgeDepartment of Biology, MIT, Cambridge, MA 02139, USA.
J Paul TaylorHoward Hughes Medical Institute, Department of Cell and Molecular Biology, St. Jude Children's Research Hospital, Memphis, TN 38105, USA.
Frank RigoIonis Pharmaceuticals, Carlsbad, CA 92010, USA.
Gene W YeoDepartment of Cellular and Molecular Medicine, University of California, San Diego, La Jolla, CA 92093, USA; Stem Cell Program and Institute for Genomic Medicine, University of California, San Diego, La Jolla, CA 92093, USA; Department of Bioinformatics and Systems Biology, University of California, San Diego, La Jolla, CA 92093, USA; Department of Physiology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore 117597, Singapore; Molecular Engineering Laboratory, A(∗)STAR, Singapore 138673, Singapore. Electronic address: geneyeo@ucsd.edu.
University of California, San Diego · USIonis Pharmaceuticals (United States) · USGladstone Institutes · USIIT@MIT · USUniversity of California, Santa Cruz · USAgency for Science, Technology and Research · SGHoward Hughes Medical Institute · USSt. Jude Children's Research Hospital · US
Funding
Comprhensive analysis of functional RNA elements encoded in the human genomeU54HG007005 · NHGRI · UNIVERSITY OF CONNECTICUT SCH OF MED/DNT · PI GRAVELEY, BRENTON R. · 2012 to 2017
$12.5MFunctional RNA elements in the human genomeR01HG004659 · NHGRI · UNIVERSITY OF CALIFORNIA, SAN DIEGO · PI Kevin Daniel Corbett, Eugene Wei-Ming Yeo · 2008 to 2026
$12.4MNeuron and Glial Cellular Signatures from Normal and Diseased iPS Cells-SupplementU54NS091046 · NINDS · UNIVERSITY OF CALIFORNIA-IRVINE · PI FINKBEINER, STEVEN M, FRAENKEL, ERNEST · 2014 to 2020
$11.7MTraining Program in Basic Clinical GeneticsT32GM008666 · NIGMS · UNIVERSITY OF CALIFORNIA, SAN DIEGO · PI HAMILTON, BRUCE A · 1998 to 2021
$8.6MDynamic RNA-protein assemblies and neurological diseaseR35NS097974 · NINDS · ST. JUDE CHILDREN'S RESEARCH HOSPITAL · PI TAYLOR, JOSEPH PAUL · 2017 to 2024
$7.2MMechanisms of human RNA turnover and quality controlR35GM118069 · NIGMS · UNIVERSITY OF CALIFORNIA, SAN DIEGO · PI LYKKE-ANDERSEN, JENS · 2016 to 2025
$5.1MGraduate Training in Computational and Systems BiologyT32GM087237 · NIGMS · MASSACHUSETTS INSTITUTE OF TECHNOLOGY · PI BURGE, CHRISTOPHER B · 2009 to 2023
$4.6MDefining the messenger RNP code in the brainR01NS075449 · NINDS · UNIVERSITY OF CALIFORNIA, SAN DIEGO · PI YEO, EUGENE WEI-MING · 2012 to 2016
$3.0MNHGRI NIH HHS R01 HG004659NHGRI NIH HHS U54 HG007005NIGMS NIH HHS R35 GM118069NIGMS NIH HHS T32 GM008666NIGMS NIH HHS T32 GM087237NINDS NIH HHS R01 NS075449NINDS NIH HHS R35 NS097974NINDS NIH HHS U54 NS091046
Abstract
HnRNPA2B1 encodes an RNA binding protein associated with neurodegeneration. However, its function in the nervous system is unclear. Transcriptome-wide crosslinking and immunoprecipitation in mouse spinal cord discover UAGG motifs enriched within ∼2,500 hnRNP A2/B1 binding sites and an unexpected role for hnRNP A2/B1 in alternative polyadenylation. HnRNP A2/B1 loss results in alternative splicing (AS), including skipping of an exon in amyotrophic lateral sclerosis (ALS)-associated D-amino acid oxidase (DAO) that reduces D-serine metabolism. ALS-associated hnRNP A2/B1 D290V mutant patient fibroblasts and motor neurons differentiated from induced pluripotent stem cells (iPSC-MNs) demonstrate abnormal splicing changes, likely due to increased nuclear-insoluble hnRNP A2/B1. Mutant iPSC-MNs display decreased survival in long-term culture and exhibit hnRNP A2/B1 localization to cytoplasmic granules as well as exacerbated changes in gene expression and splicing upon cellular stress. Our findings provide a cellular resource and reveal RNA networks relevant to neurodegeneration, regulated by normal and mutant hnRNP A2/B1. VIDEO ABSTRACT.
Indexed as
Alternative SplicingAmyotrophic Lateral SclerosisAnimalsCase-Control StudiesCell SurvivalD-Amino-Acid OxidaseFibroblastsFluorescent Antibody TechniqueGene ExpressionGene Expression ProfilingHeterogeneous-Nuclear Ribonucleoprotein Group A-BHumansInduced Pluripotent Stem CellsMiceMotor NeuronsMutationD-Amino-Acid OxidaseHeterogeneous-Nuclear Ribonucleoprotein Group A-B
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