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Guidelines

MIRAGE

asparaginyl-tRNA synthetase 2, mitochondrial

Summary

Gene Symbol

NARS2

Organism

Homo sapiens (human)

External Links

NCBI Gene

79731

PubChem

79731

Alliance of Genome Resources

HGNC:26274

JoGo

NARS2

TogoVar

NARS2

Annotation

Keyword

ATP-binding
Acetylation
Alternative splicing
Aminoacyl-tRNA synthetase
Disease variant
Leigh syndrome
Mitochondrion
Non-syndromic deafness
Protein biosynthesis
Proteomics identification
Reference proteome
Transit peptide

Proteins

Displaying **all 7** entries
UniProt	Protein Name
H0YE96
A0A8Q3SHL5
A0A8Q3WKF4
B3KPX5	Asparagine--tRNA ligase, mitochondrial
Q96I59	Asparagine--tRNA ligase, mitochondrial
Q9H5H1
A0A8Q3SHT7

Annotation information from UniProt.

Gene Ontology (GO)

Displaying **all 2** entries
GO Term	Evidence Code	PMID
asparaginyl-tRNA aminoacylation	ISS
asparaginyl-tRNA aminoacylation	IBA

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]

ncRNA metabolic process [inference]

tRNA metabolic process [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

asparaginyl-tRNA aminoacylation

organonitrogen compound metabolic process [inference]

amino acid metabolic process [inference]

amino acid activation [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

asparaginyl-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]

asparaginyl-tRNA aminoacylation

cellular aromatic compound metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

RNA metabolic process [inference]

ncRNA metabolic process [inference]

tRNA metabolic process [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

asparaginyl-tRNA aminoacylation

cellular nitrogen compound metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

RNA metabolic process [inference]

ncRNA metabolic process [inference]

tRNA metabolic process [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

asparaginyl-tRNA aminoacylation

heterocycle metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

RNA metabolic process [inference]

ncRNA metabolic process [inference]

tRNA metabolic process [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

asparaginyl-tRNA aminoacylation

primary metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

RNA metabolic process [inference]

ncRNA metabolic process [inference]

tRNA metabolic process [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

asparaginyl-tRNA aminoacylation

amino acid metabolic process [inference]

amino acid activation [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

asparaginyl-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]

asparaginyl-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]

asparaginyl-tRNA aminoacylation

macromolecule metabolic process [inference]

nucleic acid metabolic process [inference]

RNA metabolic process [inference]

ncRNA metabolic process [inference]

tRNA metabolic process [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

asparaginyl-tRNA aminoacylation

organic cyclic compound metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

RNA metabolic process [inference]

ncRNA metabolic process [inference]

tRNA metabolic process [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

asparaginyl-tRNA aminoacylation

organonitrogen compound metabolic process [inference]

amino acid metabolic process [inference]

amino acid activation [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

asparaginyl-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]

asparaginyl-tRNA aminoacylation

cellular aromatic compound metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

RNA metabolic process [inference]

ncRNA metabolic process [inference]

tRNA metabolic process [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

asparaginyl-tRNA aminoacylation

cellular nitrogen compound metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

RNA metabolic process [inference]

ncRNA metabolic process [inference]

tRNA metabolic process [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

asparaginyl-tRNA aminoacylation

heterocycle metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleic acid metabolic process [inference]

RNA metabolic process [inference]

ncRNA metabolic process [inference]

tRNA metabolic process [inference]

tRNA aminoacylation [inference]

tRNA aminoacylation for protein translation [inference]

asparaginyl-tRNA aminoacylation

Displaying entries **1 - 5** of 7 in total

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GO Term	Evidence Code	PMID
nucleoplasm	IDA
mitochondrion	ISS
mitochondrion	IDA	25807530
mitochondrion	IBA
mitochondrion	HTP	34800366

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 **all 4** entries
GO Term	Evidence Code	PMID
nucleic acid binding	IEA
asparagine-tRNA ligase activity	ISS
asparagine-tRNA ligase activity	IBA
ATP binding	IEA

GO Hierarchy

molecular function

catalytic activity [inference]

ligase activity [inference]

ligase activity, forming carbon-oxygen bonds [inference]

aminoacyl-tRNA ligase activity [inference]

asparagine-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 ligase activity [inference]

asparagine-tRNA ligase activity

binding [inference]

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]

nucleic acid binding

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

asparagine--tRNA ligase

Functional Category

G: Carbohydrate transport and metabolism
J: Translation, ribosomal structure and biogenesis
T: Signal transduction mechanisms

Displaying **all 6** entries
Gene Ontology
ATP binding
asparagine-tRNA ligase activity
asparaginyl-tRNA aminoacylation
ligase activity
nucleotide binding
tRNA aminoacylation for protein translation

Displaying **all 7** entries
InterPro
Aminoacyl-tRNA synthetase, class II (D/K/N)
Aminoacyl-tRNA synthetase, class II
Asparagine-tRNA ligase
Aspartyl/Asparaginyl-tRNA synthetase, class IIb
Class II Aminoacyl-tRNA synthetase/Biotinyl protein ligase (BPL) and lipoyl protein ligase (LPL)
Nucleic acid-binding, OB-fold
OB-fold nucleic acid binding domain, AA-tRNA synthetase-type

Disease

Disease Ontology

Displaying **all 2** entries
DO ID	Disease Name	Source
DOID:0111485	combined oxidative phosphorylation deficiency 24	Alliance of Genome Resources
DOID:0111641	autosomal recessive nonsyndromic deafness 94	Alliance of Genome Resources

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Acknowledgements

Supported by JST NBDC Grant Number JPMJND2204

Partly supported by NIH Common Fund Grant #1U01GM125267-01

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Last updated: April 6, 2026