ArticleActa neuropathologica2021
Nuclear depletion of RNA-binding protein ELAVL3 (HuC) in sporadic and familial amyotrophic lateral sclerosis.
Article in Acta neuropathologica, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed, 24 citations in OpenAlex.
- ELAVL Proteins, miRNA Fate, and Extracellular RNA Communication in Brain Aging and Neurodegeneration.Cells · 2026Review
- Full thickness three-dimensional mapping of neuronal populations within the avian enteric nervous system.Poultry science · 2026Article
- Article
- TDP-43: [GU]-ardian of the transcriptome.Molecular neurodegeneration · 2026Review
- A next-generation HDAC6 inhibitor for amyotrophic lateral sclerosis and frontotemporal dementia.Brain : a journal of neurology · 2026Article
- CircRbfox1 Contributes to Colonic Hypersensitivity in Rats With Diabetes by Altering HuC Subcellular Localization to Regulate RBFOX1 Expression.CNS neuroscience & therapeutics · 2026Article
- Hippocalcin Regulates NMDA Receptor Function and Neuronal Activity Through Elavl3 in Mouse Hippocampal Neural Precursor Cells.International journal of molecular sciences · 2026Article
- RNA-binding protein ELAVL1 modulates WDR36 to inhibit p53 pathway and reduce calcium overload in retinal cells under acute pressure elevation.Open life sciences · 2026Article
- ELAV/Hu RNA-binding protein family: key regulators in neurological disorders, cancer, and other diseases.RNA biology · 2025Review
- Machine learning-based proteomics profiling of ALS identifies downregulation of RPS29 that maintains protein homeostasis and STMN2 level.Communications biology · 2025Article
- Nonsense-mediated decay masks cryptic splicing events caused by TDP-43 loss.bioRxiv : the preprint server for biology · 2025Article
- Alternatively spliced ELAVL3 cryptic exon 4a causes ELAVL3 downregulation in ALS TDP-43 proteinopathy.Acta neuropathologica · 2024Article
- Colocalization of TDP-43 and stress granules at the early stage of TDP-43 aggregation in amyotrophic lateral sclerosis.Brain pathology (Zurich, Switzerland) · 2024Article
- The molecular genetics of nELAVL in brain development and disease.European journal of human genetics : EJHG · 2023Review
- Neuronal models of TDP-43 proteinopathy display reduced axonal translation, increased oxidative stress, and defective exocytosis.Frontiers in cellular neuroscience · 2023Article
- SCAMPR, a single-cell automated multiplex pipeline for RNA quantification and spatial mapping.Cell reports methods · 2022Article
- Aberrant NOVA1 function disrupts alternative splicing in early stages of amyotrophic lateral sclerosis.Acta neuropathologica · 2022Article
- Regulation of the Alternative Neural Transcriptome by ELAV/Hu RNA Binding Proteins.Frontiers in genetics · 2022Review
- mFrontiers in neurology · 2022Review
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Authors and funding
9 authors at 2 institutions in 1 country.
Funding
Abstract
Amyotrophic lateral sclerosis is a progressive fatal neurodegenerative disease caused by loss of motor neurons and characterized neuropathologically in almost all cases by nuclear depletion and cytoplasmic aggregation of TDP-43, a nuclear RNA-binding protein (RBP). We identified ELAVL3 as one of the most downregulated genes in our transcriptome profiles of laser captured microdissection of motor neurons from sporadic ALS nervous systems and the most dysregulated of all RBPs. Neuropathological characterizations showed ELAVL3 nuclear depletion in a great percentage of remnant motor neurons, sometimes accompanied by cytoplasmic accumulations. These abnormalities were common in sporadic cases with and without intermediate expansions in ATXN2 and familial cases carrying mutations in C9orf72 and SOD1. Depletion of ELAVL3 occurred at both the RNA and protein levels and a short protein isoform was identified, but it is not related to a TDP-43-dependent cryptic exon in intron 3. Strikingly, ELAVL3 abnormalities were more frequent than TDP-43 abnormalities and occurred in motor neurons still with normal nuclear TDP-43 present, but all neurons with abnormal TDP-43 also had abnormal ELAVL3. In a neuron-like cell culture model using SH-SY5Y cells, ELAVL3 mislocalization occurred weeks before TDP-43 abnormalities were seen. We interrogated genetic databases, but did not identify association of ELAVL3 genetic structure with ALS. Taken together, these findings suggest that ELAVL3 is an important RBP in ALS pathogenesis acquired early and the neuropathological data suggest that it is involved by loss of function rather than cytoplasmic toxicity.
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