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Article

Asparaginyl-tRNA synthetase (NARS1) variants implicated in dominant neurological phenotypes display dominant-negative properties.

HGG advances - 15 Jan 2026

Peeples Sheila M, Blake Keyana, Sutton Brendan L M, Konyukh Marina, Züchner Stephan, Stojkovic Tanya, Baets Jonathan, Antonellis Anthony

Abstract excerpt

Aminoacyl-tRNA synthetases (ARSs) are essential, ubiquitously expressed enzymes that ligate amino acids to cognate tRNAs in the cytoplasm and mitochondria. To date, seven dimeric ARS enzymes have been implicated in dominant inherited neuropathy, suggesting that tRNA charging-exacerbated by a dominant-negative effect-is a component of the peripheral nervous system (PNS) phenotype. Interestingly, heterozygosity for...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 40968538
DOI: 10.1016/j.xhgg.2025.100519
PMCID: PMC12513288

Genes

NARS1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 2 Sept 2026.

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