Asparagine–tRNA ligase, cytoplasmic

Asparagine-tRNA ligase, cytoplasmic is an enzyme that in humans is encoded by the NARS1 gene.

The NARS1 protein is an example of a Aminoacyl-tRNA synthetase, which are a class of enzymes that charge tRNAs with their cognate amino acids. Asparaginyl-tRNA synthetase is localized to the cytoplasm and belongs to the class II-b family of tRNA synthetases along with Aspartyl-tRNA synthetase and Lysyl-tRNA synthetase II. The N-terminal domain represents the signature sequence for the eukaryotic asparaginyl-tRNA synthetases.

Medical significance A particular heterozygous nonsense mutation in NARS1 is associated with a brain development disorder called neurodevelopmental disorder with microcephaly, impaired language, epilepsy, and gait abnormalities (NEDMILEG), potentially due to toxic gain-of-function. More diverse pathogenic homozygous and compound heterozygous are also associated with a syndrome with many similar symptoms called neurodevelopmental disorder with microcephaly, impaired language, and gait abnormalities (NEDMILG)

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