InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: LARS2

Summary

InnateDB Protein

IDBP-374271.4

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

LARS2

Protein Name

leucyl-tRNA synthetase 2, mitochondrial

Synonyms

LEURS; PRLTS4;

Species

Homo sapiens

Ensembl Protein

ENSP00000408576

InnateDB Gene

IDBG-29537 (LARS2)

Protein Structure

UniProt Annotation

Function

Subcellular Localization

Mitochondrion matrix {ECO:0000250}.

Disease Associations

Perrault syndrome 4 (PRLTS4) [MIM:615300]: A sex- influenced disorder characterized by sensorineural deafness in both males and females, and ovarian dysgenesis in females. Affected females have primary amenorrhea, streak gonads, and infertility, whereas affected males show normal pubertal development and are fertile. {ECO:0000269PubMed:23541342}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Ubiquitously expressed, but highest expression in tissues with high metabolic rates, such as skeletal muscle, heart, and kidney. {ECO:0000269PubMed:20194621}.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 5 experimentally validated interaction(s) in this database.

Experimentally validated
Total	5 [view]
Protein-Protein	4 [view]
Protein-DNA	1 [view]
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0000166	nucleotide binding
GO:0002161	aminoacyl-tRNA editing activity
GO:0004812	aminoacyl-tRNA ligase activity
GO:0004823	leucine-tRNA ligase activity
GO:0005524	ATP binding

Biological Process

GO:0006418	tRNA aminoacylation for protein translation
GO:0006429	leucyl-tRNA aminoacylation
GO:0006450	regulation of translational fidelity
GO:0010467	gene expression

Cellular Component

GO:0005739	mitochondrion
GO:0005759	mitochondrial matrix

Protein Structure and Domains

PDB ID

InterPro

IPR002300 Aminoacyl-tRNA synthetase, class Ia
IPR002302 Leucine-tRNA ligase
IPR009008 Valyl/Leucyl/Isoleucyl-tRNA synthetase, editing domain
IPR009080 Aminoacyl-tRNA synthetase, class 1a, anticodon-binding
IPR013155 Valyl/Leucyl/Isoleucyl-tRNA synthetase, anticodon-binding
IPR015413 Methionyl/Leucyl tRNA synthetase

PFAM

PF00133
PF08264
PF09334

PRINTS

PR00985

PIRSF

SMART

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt

Q15031