The HARS2 gene provides instructions for making an enzyme called mitochondrial histidyl-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 histidyl-tRNA synthetase, attach a particular amino acid to a specific tRNA. Mitochondrial histidyl-tRNA synthetase attaches the amino acid histidine to the correct tRNA, which helps ensure that histidine is added at the proper place in the mitochondrial protein.
Other Names
HARS-related; HARSL; HARSR; hisRS; histidine translase; histidine tRNA ligase 2, mitochondrial (putative); histidine-tRNA ligase homolog; histidyl-tRNA synthetase 2; histidyl-tRNA synthetase 2, mitochondrial (putative); HO3; PRLTS2; probable histidine–tRNA ligase, mitochondrial; probable histidine–tRNA ligase, mitochondrial isoform 2; probable histidine–tRNA ligase, mitochondrial isoform 3; probable histidyl-tRNA synthetase, mitochondrial
Related Genetics Topics
Identifiers
- NCBI Gene: 23438
- OMIM: 600783
Source: MedlinePlus Genetics, U.S. National Library of Medicine.
