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Article

Dominant-negative isoform of TDP-43 is regulated by ALS-linked RNA-binding proteins

24 Jun 2025

Abstract excerpt

TDP-43, an RNA-binding protein (RBP) encoded by the TARDBP gene, is crucial for understanding the pathogenesis of neurodegenerative diseases like amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration. Dysregulated TDP-43 causes motor neuron loss, highlighting the need for proper expression levels. Here, we identify a dominant-negative isoform among the multiple TARDBP splicing variants and...

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PMID: 40778857
DOI: 10.1083/jcb.202406097

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