// Workers AI · dad joke modeWhy did Threonine–tRNA ligase go to therapy? It had an amino acid to work through.

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.^[5]^[6] Like the related cytosolic threonine–tRNA ligase, TARS1, this enzyme functions to attach the amino acid threonine to its corresponding transfer RNA (tRNA^Thr).^[6] This produces an aminoacyl-tRNA which is needed to incorporate threonine into proteins during RNA-to-protein translation.^[7]

Function

This enzyme is part of the class-II aminoacyl-tRNA synthetase family.^[5] 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.^[7] During translation, the produced aminoacyl-tRNAs are used to translate the mRNA code into the corresponding amino acid sequence and protein.^[7]

Medical Significance

Pathogenic mutations in TARS2 are associated with a growth defects, seizures, and early death, called combined oxidative phosphorylation deficiency-21 (COXPD21).^[8] This illness appears to follow an autosomal recessive inheritance pattern.^[8]

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. doi:10.1261/rna.071720.119. PMC 7373986.
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. doi:10.1261/rna.071720.119. PMC 7373986.

[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.

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