Predicted to enable leucine-tRNA ligase activity. Predicted to be involved in leucyl-tRNA aminoacylation and mitochondrial translation. Predicted to be active in mitochondrion. Is expressed in body wall musculature; intestine; and neurons. Used to study Perrault syndrome. Human ortholog(s) of this gene implicated in Perrault syndrome. Orthologous to human LARS2 (leucyl-tRNA synthetase 2, mitochondrial).
WB Description
lars-2 encodes the ortholog of human leucyl amino-acyl tRNA synthetase (LARS); in C. elegans, lars-2 is required for normal mitochondrial structure and function, and acts downstream of daf-2 to control lifespan; lars-2 mutants show swollen, disorganized and sometimes fused mitochondria, and live much longer than wild-type worms; a lars-2 GFP translation fusion gene was ubiquitously expressed, with prominent expression in body-wall muscle and neurons and with subcellular localization in the mitochondria; being a tRNA synthetase that charges mitochondrial tRNAs for translation, lars-2 is predicted to specifically compromise mitochondrial electron transport and ATP synthesis, through it's effects on mitochrondrially encoded genes.