The LARS2 gene encodes mitochondrial leucyl-tRNA synthetase, an enzyme that belongs to the class I aminoacyl-tRNA synthetase group of proteins. During mitochondrial protein translation, leucyl-tRNA synthetase is responsible for attaching the amino acid leucine to the appropriate tRNA. As a mitochondrial enzyme, LARS2 is believed to play an important role in mitochondrial energy production.
The gene has been linked to Perrault Syndrome 4, an autosomal recessive disorder characterized by hearing loss and ovarian failure. The gene is also associated with Hydrops, Lactic Acidosis, and Sideroblastic Anemia (HLASA), a fatal multisystem disorder defined by cardiovascular, neurologic, hepatic, respiratory and metabolic irregularities.