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Guidelines

MIRAGE

threonyl-tRNA synthetase 2, mitochondrial

Summary

Gene Symbol

TARS2

Organism

Homo sapiens (human)

External Links

NCBI Gene

80222

PubChem

80222

Alliance of Genome Resources

HGNC:30740

Annotation

Keyword

ATP-binding
Alternative splicing
Aminoacyl-tRNA synthetase
Disease variant
Mitochondrion
Phosphoprotein
Primary mitochondrial disease
Protein biosynthesis
Proteomics identification
Reference proteome
Transit peptide

Proteins

Displaying **all 2** entries
UniProt	Protein Name
U3KQG0	Threonyl-tRNA synthetase Threonyl-tRNA synthetase-like 1
Q9BW92	Threonyl-tRNA synthetase Threonyl-tRNA synthetase-like 1

Annotation information from UniProt.

Gene Ontology (GO)

Displaying **all 3** entries
GO Term	Evidence Code	PMID
threonyl-tRNA aminoacylation	IBA
mitochondrial threonyl-tRNA aminoacylation	TAS	15779907
aminoacyl-tRNA metabolism involved in translational fidelity	IEA

GO Hierarchy

biological process

metabolic process [inference]

nitrogen compound metabolic process [inference]

cellular nitrogen compound metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

RNA metabolic process [inference]

mitochondrial RNA metabolic process [inference]

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

ncRNA metabolic process [inference]

tRNA metabolic process [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

threonyl-tRNA aminoacylation

mitochondrial threonyl-tRNA aminoacylation

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

aminoacyl-tRNA metabolism involved in translational fidelity

organonitrogen compound metabolic process [inference]

amino acid metabolic process [inference]

amino acid activation [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

threonyl-tRNA aminoacylation

mitochondrial threonyl-tRNA aminoacylation

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

cellular metabolic process [inference]

organic acid metabolic process [inference]

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

amino acid metabolic process [inference]

amino acid activation [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

threonyl-tRNA aminoacylation

mitochondrial threonyl-tRNA aminoacylation

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

cellular aromatic compound metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

RNA metabolic process [inference]

mitochondrial RNA metabolic process [inference]

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

ncRNA metabolic process [inference]

tRNA metabolic process [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

threonyl-tRNA aminoacylation

mitochondrial threonyl-tRNA aminoacylation

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

aminoacyl-tRNA metabolism involved in translational fidelity

cellular nitrogen compound metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

RNA metabolic process [inference]

mitochondrial RNA metabolic process [inference]

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

ncRNA metabolic process [inference]

tRNA metabolic process [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

threonyl-tRNA aminoacylation

mitochondrial threonyl-tRNA aminoacylation

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

aminoacyl-tRNA metabolism involved in translational fidelity

heterocycle metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

RNA metabolic process [inference]

mitochondrial RNA metabolic process [inference]

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

ncRNA metabolic process [inference]

tRNA metabolic process [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

threonyl-tRNA aminoacylation

mitochondrial threonyl-tRNA aminoacylation

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

aminoacyl-tRNA metabolism involved in translational fidelity

primary metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

RNA metabolic process [inference]

mitochondrial RNA metabolic process [inference]

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

ncRNA metabolic process [inference]

tRNA metabolic process [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

threonyl-tRNA aminoacylation

mitochondrial threonyl-tRNA aminoacylation

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

aminoacyl-tRNA metabolism involved in translational fidelity

amino acid metabolic process [inference]

amino acid activation [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

threonyl-tRNA aminoacylation

mitochondrial threonyl-tRNA aminoacylation

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

small molecule metabolic process [inference]

organic acid metabolic process [inference]

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

amino acid metabolic process [inference]

amino acid activation [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

threonyl-tRNA aminoacylation

mitochondrial threonyl-tRNA aminoacylation

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

organic substance metabolic process [inference]

organic acid metabolic process [inference]

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

amino acid metabolic process [inference]

amino acid activation [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

threonyl-tRNA aminoacylation

mitochondrial threonyl-tRNA aminoacylation

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

macromolecule metabolic process [inference]

nucleic acid metabolic process [inference]

RNA metabolic process [inference]

mitochondrial RNA metabolic process [inference]

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

ncRNA metabolic process [inference]

tRNA metabolic process [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

threonyl-tRNA aminoacylation

mitochondrial threonyl-tRNA aminoacylation

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

aminoacyl-tRNA metabolism involved in translational fidelity

organic cyclic compound metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

RNA metabolic process [inference]

mitochondrial RNA metabolic process [inference]

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

ncRNA metabolic process [inference]

tRNA metabolic process [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

threonyl-tRNA aminoacylation

mitochondrial threonyl-tRNA aminoacylation

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

aminoacyl-tRNA metabolism involved in translational fidelity

organonitrogen compound metabolic process [inference]

amino acid metabolic process [inference]

amino acid activation [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

threonyl-tRNA aminoacylation

mitochondrial threonyl-tRNA aminoacylation

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

cellular process [inference]

cellular metabolic process [inference]

organic acid metabolic process [inference]

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

amino acid metabolic process [inference]

amino acid activation [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

threonyl-tRNA aminoacylation

mitochondrial threonyl-tRNA aminoacylation

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

cellular aromatic compound metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

RNA metabolic process [inference]

mitochondrial RNA metabolic process [inference]

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

ncRNA metabolic process [inference]

tRNA metabolic process [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

threonyl-tRNA aminoacylation

mitochondrial threonyl-tRNA aminoacylation

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

aminoacyl-tRNA metabolism involved in translational fidelity

cellular nitrogen compound metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

RNA metabolic process [inference]

mitochondrial RNA metabolic process [inference]

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

ncRNA metabolic process [inference]

tRNA metabolic process [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

threonyl-tRNA aminoacylation

mitochondrial threonyl-tRNA aminoacylation

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

aminoacyl-tRNA metabolism involved in translational fidelity

heterocycle metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

RNA metabolic process [inference]

mitochondrial RNA metabolic process [inference]

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

ncRNA metabolic process [inference]

tRNA metabolic process [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

threonyl-tRNA aminoacylation

mitochondrial threonyl-tRNA aminoacylation

tRNA aminoacylation for mitochondrial protein translation [inference]

mitochondrial threonyl-tRNA aminoacylation

aminoacyl-tRNA metabolism involved in translational fidelity

biological regulation [inference]

regulation of biological quality [inference]

regulation of translational fidelity [inference]

aminoacyl-tRNA metabolism involved in translational fidelity

Displaying **all 3** entries
GO Term	Evidence Code	PMID
mitochondrion	IBA
mitochondrion	HTP	34800366
mitochondrial matrix	IEA

GO Hierarchy

cellular component

cellular anatomical entity [inference]

membrane-enclosed lumen [inference]

organelle lumen [inference]

intracellular organelle lumen [inference]

mitochondrial matrix

organelle [inference]

membrane-bounded organelle [inference]

intracellular membrane-bounded organelle [inference]

mitochondrion

intracellular organelle [inference]

intracellular membrane-bounded organelle [inference]

mitochondrion

Displaying entry **6 - 6** of 6 in total

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GO Term	Evidence Code	PMID
protein homodimerization activity	IDA	26811336

GO Hierarchy

molecular function

catalytic activity [inference]

hydrolase activity [inference]

hydrolase activity, acting on ester bonds [inference]

carboxylic ester hydrolase activity [inference]

aminoacyl-tRNA editing activity

ligase activity [inference]

ligase activity, forming carbon-oxygen bonds [inference]

aminoacyl-tRNA ligase activity [inference]

threonine-tRNA ligase activity

catalytic activity, acting on a nucleic acid [inference]

catalytic activity, acting on RNA [inference]

catalytic activity, acting on a tRNA [inference]

aminoacyl-tRNA editing activity

aminoacyl-tRNA ligase activity [inference]

threonine-tRNA ligase activity

binding [inference]

protein binding

identical protein binding [inference]

protein homodimerization activity

protein dimerization activity [inference]

protein homodimerization activity

small molecule binding [inference]

heterocyclic compound binding [inference]

nucleotide binding [inference]

purine nucleotide binding [inference]

adenyl nucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

ion binding [inference]

anion binding [inference]

purine ribonucleoside triphosphate binding [inference]

ATP binding

organic cyclic compound binding [inference]

nucleoside phosphate binding [inference]

nucleotide binding [inference]

purine nucleotide binding [inference]

adenyl nucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

purine ribonucleoside triphosphate binding [inference]

ATP binding

carbohydrate derivative binding [inference]

ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

Gene Ontology information from NCBI Gene and UniProt.

OrthoDB (Group)

Group level

Eukaryota

Group Name

Threonine--trna ligase

Functional Category

J: Translation, ribosomal structure and biogenesis
K: Transcription
T: Signal transduction mechanisms

Displaying **all 7** entries
Gene Ontology
ATP binding
ligase activity
nucleotide binding
tRNA aminoacylation for protein translation
tRNA aminoacylation
threonine-tRNA ligase activity
threonyl-tRNA aminoacylation

Displaying entries **1 - 10** of 13 in total

1

2

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InterPro
Aminoacyl-tRNA synthetase, class II (G/ P/ S/T)
Aminoacyl-tRNA synthetase, class II
Anticodon-binding domain superfamily
Anticodon-binding
Beta-grasp domain superfamily
Class II Aminoacyl-tRNA synthetase/Biotinyl protein ligase (BPL) and lipoyl protein ligase (LPL)
TGS-like
TGS
Threonine-tRNA ligase catalytic core domain
Threonine-tRNA ligase, class IIa, anticodon-binding domain

Disease

Disease Ontology

Displaying 1 entry
DO ID	Disease Name	Source
DOID:0111465	combined oxidative phosphorylation deficiency 21	Alliance of Genome Resources

Ortholog

Displaying entries **11 - 20** of 44 in total

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Species	Gene ID	Alliance of Genome Resources	Orthologous MAtrix
Monodelphis domestica	100017092		MONDO15288
Equus caballus	100059019		HORSE34899
Callithrix jacchus	100404961		CALJA15762
Pongo abelii	100437585		PONAB18175
Ailuropoda melanoleuca	100468748		AILME12689
Nomascus leucogenys	100602917		NOMLE05471
Loxodonta africana	100676882		LOXAF15201
Cricetulus griseus	100762707		CRIGR05056
Pan paniscus	100978264		PANPA02344
Papio anubis	101016485		PAPAN18430

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Last updated: December 8, 2025