Threonine–tRNA ligase, mitochondrial

TARS2
Identifiers
Aliases	TARS2, COXPD21, TARSL1, thrRS, threonyl-tRNA synthetase 2, mitochondrial (putative), threonyl-tRNA synthetase 2, mitochondrial
External IDs	OMIM: 612805; MGI: 1919057; GeneCards: TARS2
Enzyme activity
Gene location (Human)
Chr.	Chromosome 1 (human)
End	150,507,602 bp
Gene location (Mouse)
Chr.	Chromosome 3 (mouse)
End	95,667,518 bp
RNA expression pattern
	Top expressed in
	gastrocnemius muscle; ; apex of heart; ; muscle of thigh; ; cerebellar vermis; ; triceps brachii muscle; ; right adrenal gland; ; left ventricle; ; right hemisphere of cerebellum; ; buccal mucosa cell; ; right lobe of liver;
	Top expressed in
	ectoderm; ; otic vesicle; ; otic placode; ; saccule; ; spermatocyte; ; epiblast; ; blastocyst; ; crypt of lieberkuhn of small intestine; ; motor neuron; ; Paneth cell;
	More reference expression data
	n/a
Gene ontology
Molecular function	aminoacyl-tRNA ligase activity; nucleotide binding; ligase activity; ATP binding; aminoacyl-tRNA editing activity; protein homodimerization activity; threonine-tRNA ligase activity;
Cellular component	cytoplasm; mitochondrial matrix; mitochondrion; cellular component;
Biological process	mitochondrial threonyl-tRNA aminoacylation; threonyl-tRNA aminoacylation; tRNA aminoacylation; protein biosynthesis; tRNA aminoacylation for protein translation; aminoacyl-tRNA metabolism involved in translational fidelity;
	Sources:Amigo / QuickGO
Orthologs
Databases	NCBI: entry; OMA: entry
	80222
	71807
	ENSG00000143374
	ENSMUSG00000028107
	Q9BW92
	Q3UQ84
	NM_001271895; NM_001271896; NM_025150
NM_001163617; NM_001163618; NM_001163619; NM_027931; NM_001356965;
	NM_001356966; NM_001356967
	NP_001258824; NP_001258825; NP_079426
NP_001157089; NP_001157090; NP_001157091; NP_082207; NP_001343894;
	NP_001343895; NP_001343896
	Wikidata
View/Edit Human	View/Edit Mouse

Threonine–tRNA ligase, mitochondrial, also called threonyl-tRNA synthetase 2, is an enzyme that in humans is encoded by the TARS2 gene. Like the related cytosolic threonine–tRNA ligase, TARS1, this enzyme functions to attach the amino acid threonine to its corresponding transfer RNA (tRNA^Thr). This produces an aminoacyl-tRNA which is needed to incorporate threonine into proteins during RNA-to-protein translation.

Function

This enzyme is part of the class-II aminoacyl-tRNA synthetase family. Aminoacyl tRNA synthetases are enzymes that charge tRNA with the amino acids of the appropriate type, which in this case means attaching threonine to tRNA^Thr. During translation, the produced aminoacyl-tRNAs are used to translate the mRNA code into the corresponding amino acid sequence and protein.

Medical Significance

Pathogenic mutations in TARS2 are associated with a growth defects, seizures, and early death, called combined oxidative phosphorylation deficiency-21 (COXPD21). This illness appears to follow an autosomal recessive inheritance pattern.

References

1 2 3 GRCh38: Ensembl release 89: ENSG00000143374 – Ensembl, May 2017
1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000028107 – 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.
1 2 "TARS2 threonyl-tRNA synthetase 2, mitochondrial". Entrez Gene. NCBI. Retrieved 2026-09-30.
1 2 "Threonine--tRNA ligase, mitochondrial". uniprot.org. UniProt consortium. Retrieved 2026-09-30.
1 2 3 Gomez MA, Ibba M (2020-04-17). "Aminoacyl-tRNA synthetases". RNA. 26 (8): 910–936. doi:10.1261/rna.071720.119. PMC 7373986. PMID 32303649.
1 2 Kniffin CL, Vernon HJ (2025-03-25). "COMBINED OXIDATIVE PHOSPHORYLATION DEFICIENCY 21; COXPD21". Online Mendelian Inheritance in Man. Johns Hopkins University. Retrieved 2026-09-30.

This article on a gene on human chromosome 1 is a stub. You can help Wikipedia by adding missing information.

[refGRCh38Ensembl-1] 1 2 3 GRCh38: Ensembl release 89: ENSG00000143374 – Ensembl, May 2017

[refGRCm38Ensembl-2] 1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000028107 – 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.

[entrez-5] 1 2 "TARS2 threonyl-tRNA synthetase 2, mitochondrial". Entrez Gene. NCBI. Retrieved 2026-09-30.

[uniprot-6] 1 2 "Threonine--tRNA ligase, mitochondrial". uniprot.org. UniProt consortium. Retrieved 2026-09-30.

[Gomez2020-7] 1 2 3 Gomez MA, Ibba M (2020-04-17). "Aminoacyl-tRNA synthetases". RNA. 26 (8): 910–936. doi:10.1261/rna.071720.119. PMC 7373986. PMID 32303649.

[omim-8] 1 2 Kniffin CL, Vernon HJ (2025-03-25). "COMBINED OXIDATIVE PHOSPHORYLATION DEFICIENCY 21; COXPD21". Online Mendelian Inheritance in Man. Johns Hopkins University. Retrieved 2026-09-30.