Asparagine–tRNA ligase, cytoplasmic

NARS1
Identifiers
Aliases	NARS1, NARS, NEDMILG, NEDMILEG, ASNRS, asparaginyl-tRNA synthetase 1, asparaginyl-tRNA synthetase
External IDs	OMIM: 108410; MGI: 1917473; GeneCards: NARS1
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	4ZYA
Enzyme activity
Gene location (Human)
Chr.	Chromosome 18 (human)
End	57,622,213 bp
Gene location (Mouse)
Chr.	Chromosome 18 (mouse)
End	64,649,723 bp
RNA expression pattern
	Top expressed in
	endothelial cell; ; pancreatic ductal cell; ; Brodmann area 23; ; lateral nuclear group of thalamus; ; middle temporal gyrus; ; kidney tubule; ; gingival epithelium; ; mucosa of sigmoid colon; ; parotid gland; ; Epithelium of choroid plexus;
	Top expressed in
	facial motor nucleus; ; Paneth cell; ; motor neuron; ; lacrimal gland; ; gastrula; ; calvaria; ; seminal vesicula; ; anterior horn of spinal cord; ; parotid gland; ; decidua;
	More reference expression data
	; ; ; ;
	More reference expression data
Gene ontology
Molecular function	aminoacyl-tRNA ligase activity; nucleotide binding; ligase activity; asparagine-tRNA ligase activity; ATP binding; nucleic acid binding;
Cellular component	extracellular exosome; mitochondrion; cytoplasm; cytosol;
Biological process	protein biosynthesis; asparaginyl-tRNA aminoacylation; tRNA aminoacylation for protein translation;
	Sources:Amigo / QuickGO
Orthologs
Databases	NCBI: entry; OMA: entry
	4677
	70223
	ENSG00000134440
	ENSMUSG00000024587
	O43776
	Q8BP47
	NM_004539
	NM_001142950; NM_027350; NM_001377021; NM_001377022; NM_001377023
	NP_004530
	NP_001136422; NP_081626
	Wikidata
View/Edit Human	View/Edit Mouse

Asparagine–tRNA ligase, cytoplasmic, also called asparaginyl-tRNA synthetase 1, is an enzyme that in humans is encoded by the NARS1 gene. NARS1 functions to attach the amino acid asparagine to its corresponding transfer RNA (tRNA^Asn), which is important for incorporating asparagine into proteins during protein synthesis.

Function

The NARS1 protein is an example of an aminoacyl-tRNA synthetase, which are a class of enzymes that charge tRNAs with their cognate amino acids, in this case attaching asparagine to tRNA^Asn to form the aminoacyl-tRNA, L-asparaginyl-tRNA^Asn. This enzyme typically binds with another copy of itself to form a homodimer, and does not appear to be part of the multi-synthetase complex.

In humans, NARS1 is particularly important in the development of the cerebral cortex, where it is required for the proliferation of radial glial cells. Outside of its core role in protein synthesis, it can also act as a signaling molecule which is able to induce the migration of CCR3-expressing cells.

Family

Cytoplasmic asparaginyl-tRNA synthetase 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

Certain heterozygous mutations in NARS1 are associated with a brain development and neurological disorder called neurodevelopmental disorder with microcephaly, impaired language, epilepsy, and gait abnormalities (NEDMILEG). 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)

References

1 2 3 GRCh38: Ensembl release 89: ENSG00000134440 – Ensembl, May 2017
1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000024587 – Ensembl, May 2017
↑ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
↑ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
↑ Cirullo RE, Arredondo-Vega FX, Smith M, Wasmuth JJ (May 1983). "Isolation and characterization of interspecific heat-resistant hybrids between a temperature-sensitive chinese hamster cell asparaginyl-tRNA synthetase mutant and normal human leukocytes: assignment of human asnS gene to chromosome 18". Somatic Cell Genet. 9 (2): 215–33. doi:10.1007/BF01543178. PMID 6836455. S2CID 42353500.
↑ Beaulande M, Tarbouriech N, Hartlein M (Feb 1998). "Human cytosolic asparaginyl-tRNA synthetase: cDNA sequence, functional expression in Escherichia coli and characterization as human autoantigen". Nucleic Acids Res. 26 (2): 521–4. doi:10.1093/nar/26.2.521. PMC 147268. PMID 9421509.
1 2 3 "Entrez Gene: NARS asparaginyl-tRNA synthetase".
1 2 3 4 5 "Asparagine--tRNA ligase, cytoplasmic". UniProt consortium. Retrieved 2026-10-17.
↑ Samuels TN, Wu F, Mahmood M, Abuzaid WA, Sun N, Moresco A, Siu VM, O'Donoghue P, Heinemann IU (June 2025). "Transfer RNA and small molecule therapeutics for aminoacyl‐tRNA synthetase diseases". The FEBS Journal. 292 (11): 2737–50. doi:10.1111/febs.17361.
↑ "NEURODEVELOPMENTAL DISORDER WITH MICROCEPHALY, IMPAIRED LANGUAGE, EPILEPSY, AND GAIT ABNORMALITIES; NEDMILEG". Online Mendelian Inheritance in Man. Johns Hopkins University. Retrieved 2026-09-27.
↑ "NEURODEVELOPMENTAL DISORDER WITH MICROCEPHALY, IMPAIRED LANGUAGE, AND GAIT ABNORMALITIES; NEDMILG". Online Mendelian Inheritance in Man. Johns Hopkins University. Retrieved 2026-09-27.

Maruyama K, Sugano S (1994). "Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides". Gene. 138 (1–2): 171–4. doi:10.1016/0378-1119(94)90802-8. PMID 8125298.
Andersson B, Wentland MA, Ricafrente JY, et al. (1996). "A "double adaptor" method for improved shotgun library construction". Anal. Biochem. 236 (1): 107–13. doi:10.1006/abio.1996.0138. PMID 8619474.
Yu W, Andersson B, Worley KC, et al. (1997). "Large-scale concatenation cDNA sequencing". Genome Res. 7 (4): 353–8. doi:10.1101/gr.7.4.353. PMC 139146. PMID 9110174.
Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K, et al. (1997). "Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library". Gene. 200 (1–2): 149–56. doi:10.1016/S0378-1119(97)00411-3. PMID 9373149.
Shiba K, Motegi H, Yoshida M, Noda T (1999). "Human asparaginyl-tRNA synthetase: molecular cloning and the inference of the evolutionary history of Asx-tRNA synthetase family". Nucleic Acids Res. 26 (22): 5045–51. doi:10.1093/nar/26.22.5045. PMC 147956. PMID 9801298.
Strausberg RL, Feingold EA, Grouse LH, et al. (2003). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–903. Bibcode:2002PNAS...9916899M. doi:10.1073/pnas.242603899. PMC 139241. PMID 12477932.
Lehner B, Sanderson CM (2004). "A protein interaction framework for human mRNA degradation". Genome Res. 14 (7): 1315–23. doi:10.1101/gr.2122004. PMC 442147. PMID 15231747.
Gerhard DS, Wagner L, Feingold EA, et al. (2004). "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)". Genome Res. 14 (10B): 2121–7. doi:10.1101/gr.2596504. PMC 528928. PMID 15489334.
Rual JF, Venkatesan K, Hao T, et al. (2005). "Towards a proteome-scale map of the human protein-protein interaction network". Nature. 437 (7062): 1173–8. Bibcode:2005Natur.437.1173R. doi:10.1038/nature04209. PMID 16189514. S2CID 4427026.
Lim J, Hao T, Shaw C, et al. (2006). "A protein-protein interaction network for human inherited ataxias and disorders of Purkinje cell degeneration". Cell. 125 (4): 801–14. doi:10.1016/j.cell.2006.03.032. PMID 16713569. S2CID 13709685.

This article on a gene on human chromosome 18 is a stub. You can help Wikipedia by adding missing information.

[refGRCh38Ensembl-1] 1 2 3 GRCh38: Ensembl release 89: ENSG00000134440 – Ensembl, May 2017

[refGRCm38Ensembl-2] 1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000024587 – Ensembl, May 2017

[3] "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[4] "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[pmid6836455-5] Cirullo RE, Arredondo-Vega FX, Smith M, Wasmuth JJ (May 1983). "Isolation and characterization of interspecific heat-resistant hybrids between a temperature-sensitive chinese hamster cell asparaginyl-tRNA synthetase mutant and normal human leukocytes: assignment of human asnS gene to chromosome 18". Somatic Cell Genet. 9 (2): 215–33. doi:10.1007/BF01543178. PMID 6836455. S2CID 42353500.

[pmid9421509-6] Beaulande M, Tarbouriech N, Hartlein M (Feb 1998). "Human cytosolic asparaginyl-tRNA synthetase: cDNA sequence, functional expression in Escherichia coli and characterization as human autoantigen". Nucleic Acids Res. 26 (2): 521–4. doi:10.1093/nar/26.2.521. PMC 147268. PMID 9421509.

[entrez-7] 1 2 3 "Entrez Gene: NARS asparaginyl-tRNA synthetase".

[uniprot-8] 1 2 3 4 5 "Asparagine--tRNA ligase, cytoplasmic". UniProt consortium. Retrieved 2026-10-17.

[9] Samuels TN, Wu F, Mahmood M, Abuzaid WA, Sun N, Moresco A, Siu VM, O'Donoghue P, Heinemann IU (June 2025). "Transfer RNA and small molecule therapeutics for aminoacyl‐tRNA synthetase diseases". The FEBS Journal. 292 (11): 2737–50. doi:10.1111/febs.17361.

[10] "NEURODEVELOPMENTAL DISORDER WITH MICROCEPHALY, IMPAIRED LANGUAGE, EPILEPSY, AND GAIT ABNORMALITIES; NEDMILEG". Online Mendelian Inheritance in Man. Johns Hopkins University. Retrieved 2026-09-27.

[11] "NEURODEVELOPMENTAL DISORDER WITH MICROCEPHALY, IMPAIRED LANGUAGE, AND GAIT ABNORMALITIES; NEDMILG". Online Mendelian Inheritance in Man. Johns Hopkins University. Retrieved 2026-09-27.

Asparagine–tRNA ligase, cytoplasmic

Function

Family

Medical significance

See also

References

Further reading