Recombinant Human Leucine–tRNA ligase, cytoplasmic (LARS1), Truncated

Uniprot Id

Q9P2J5

Target Species

Human

Target Name

LARS1

Target Full Name

Leucine--tRNA ligase, cytoplasmic

Target Function

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.

Target Involvement

Infantile liver failure syndrome 1 (ILFS1)

Target Subcellular Location

Cytoplasm.

Target Protein Families

Class-I aminoacyl-tRNA synthetase family

Target Synonyms

cytoplasmic; Cytoplasmic leucyl tRNA synthetase; Cytosolic leucyl tRNA synthetase; FLJ10595; FLJ21788; hr025Cl; HSPC 192; HSPC192; KIAA1352; LARS 1; Lars; LARS1; Leucine translase; Leucine tRNA ligase; Leucine--tRNA ligase; Leucyl tRNA synthetase cytoplasmic; Leucyl-tRNA synthetase; LeuRS; LEUS; LRS; PIG 44; PIG44; Proliferation inducing gene 44; RNTLS; SYLC_HUMAN

Target Background

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.