Publications by authors named "Kevin M Kim"
Arginine-rich dipeptide repeat proteins (R-DPRs) are highly toxic proteins found in patients with C9orf72-linked amyotrophic lateral sclerosis and frontotemporal dementia (C9-ALS/FTD). R-DPRs can cause toxicity by disrupting the natural phase behavior of RNA-binding proteins (RBPs). Mitigating this abnormal phase behavior is, therefore, crucial to reduce R-DPR-induced toxicity.
View Article and Find Full Text PDFImportin β-superfamily nuclear import receptors (NIRs) mitigate mislocalization and aggregation of RNA-binding proteins (RBPs), like FUS and TDP-43, which are implicated in neurodegenerative diseases. NIRs potently disaggregate RBPs by recognizing their nuclear localization signal (NLS). However, disease-causing mutations in NLS compromise NIR binding and activity.
View Article and Find Full Text PDFArticle Synopsis
- * Short RNAs (24-48 nucleotides) can prevent FUS from forming pathological aggregates and even reverse its harmful condensation by interacting with different RNA-binding domains.
- * A specific short RNA effectively breaks down harmful aggregates of FUS and TDP-43, restoring their normal function in neurons, suggesting potential for RNA-based therapies for ALS and FTD.