Aliases for LARS1 Gene
External Ids for LARS1 Gene
Previous HGNC Symbols for LARS1 Gene
This gene encodes a cytosolic leucine-tRNA synthetase, a member of the class I aminoacyl-tRNA synthetase family. The encoded enzyme catalyzes the ATP-dependent ligation of L-leucine to tRNA(Leu). It is found in the cytoplasm as part of a multisynthetase complex and interacts with the arginine tRNA synthetase through its C-terminal domain. A mutation in this gene was found in affected individuals with infantile liver failure syndrome 1. Alternatively spliced transcript variants of this gene have been observed. [provided by RefSeq, Dec 2015]
GeneCards Summary for LARS1 Gene
LARS1 (Leucyl-TRNA Synthetase 1) is a Protein Coding gene. Diseases associated with LARS1 include Infantile Liver Failure Syndrome 1 and Infantile Liver Failure Syndrome. Among its related pathways are Viral mRNA Translation and tRNA Aminoacylation. An important paralog of this gene is VARS2.
UniProtKB/Swiss-Prot Summary for LARS1 Gene
Catalyzes the specific attachment of an amino acid to its cognate tRNA in a two step reaction: the amino acid (AA) is first activated by ATP to form AA-AMP and then transferred to the acceptor end of the tRNA. Exhibits a post-transfer editing activity to hydrolyze mischarged tRNAs.