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Updated: Dec 8, 2025
Inducible and Reversible Dominant-negative DN Protein Inhibition
Published on: January 7, 2019
Evidence for a dominant-negative mechanism in HARS1-mediated peripheral neuropathy
Rebecca Meyer-Schuman1, Anthony Antonellis1,2
1Department of Human Genetics, University of Michigan, Ann Arbor, MI, USA.
Abstract:
The pathogenic mechanism of neuropathy-associated aminoacyl-tRNA synthetase (ARS) gene variants is poorly defined. Mullen et al. generate new models of pathogenic, dominant HARS1 mutations and show that they increase eIF2α phosphorylation and decrease protein translation in neurons. These results are consistent with a dominant-negative mechanism of ARS-mediated peripheral neuropathy. Comment on: https://doi.org/10.1111/febs.15449.
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