Alanyl-tRNA synthetase domain containing 1 (AARSD1)

Alanyl-tRNA synthetase domain containing 1 (AARSD1) is an enzyme involved in maintaining the accuracy of protein synthesis. It specifically edits charged tRNA by removing serine from incorrectly charged Ser-tRNA(Ala), protecting cells from misincorporation of amino acids—a process crucial for protein quality control and cellular homeostasis. AARSD1 belongs to the class-II aminoacyl-tRNA synthetase family and is part of the group of proteins responsible for high-fidelity translation by correcting errors in tRNA aminoacylation. Mutations in AARSD1 are associated with certain hereditary neuronal disorders, notably autosomal recessive distal hereditary motor neuronopathy. Its principal function involves deacylase activity localized to the nucleus, emphasizing its role in preventing translational errors. No drugs currently target AARSD1 directly, and there are no identified biomarkers or significant safety concerns related to manipulating its activity. The nomenclature, aliases, and functional attributes described here conform to the latest curated gene and protein databases and scientific literature.

Other names

Alanyl-tRNA editing protein Aarsd1AlaXpMGC2744alanyl-tRNA synthetase domain-containing protein 1

Not applicable—there are no clinically relevant drugs targeting AARSD1 at present