The LARS2 gene provides instructions for making an enzyme called mitochondrial leucyl-tRNA synthetase. This enzyme is important in the production (synthesis) of proteins in cellular structures called mitochondria, the energy-producing centers in cells. While most protein synthesis occurs in the fluid surrounding the nucleus (cytoplasm), some proteins are synthesized in the mitochondria.
During protein synthesis, in either the mitochondria or the cytoplasm, a type of RNA called transfer RNA (tRNA) helps assemble protein building blocks (amino acids) into a chain that forms the protein. Each tRNA carries a specific amino acid to the growing chain. Enzymes called aminoacyl-tRNA synthetases, including mitochondrial leucyl-tRNA synthetase, attach a particular amino acid to a specific tRNA. Mitochondrial leucyl-tRNA synthetase attaches the amino acid leucine to the correct tRNA, which helps ensure that leucine is added at the proper place in the mitochondrial protein.
Other Names
KIAA0028; leucine translase; leucine tRNA ligase 2, mitochondrial; leucyl-tRNA synthetase 2; LEURS; MGC26121; mtLeuRS; PRLTS4; probable leucine–tRNA ligase, mitochondrial; probable leucyl-tRNA synthetase, mitochondrial
Related Genetics Topics
Identifiers
- NCBI Gene: 23395
- OMIM: 604544
Source: MedlinePlus Genetics, U.S. National Library of Medicine.
