HARS2 : histidyl-tRNA synthetase 2, mitochondrial


Description

The HARS2 (histidyl-tRNA synthetase 2, mitochondrial) is a protein-coding gene located on chromosome 5.

The HARS2 gene provides instructions for making an enzyme called mitochondrial histidyl-tRNA synthetase. This enzyme is crucial for protein synthesis within mitochondria, the energy-producing centers of cells. While most protein synthesis occurs in the cytoplasm, mitochondria have their own protein production machinery. During protein synthesis, transfer RNA (tRNA) molecules carry specific amino acids to the growing protein chain. Aminoacyl-tRNA synthetases, like mitochondrial histidyl-tRNA synthetase, attach the correct amino acid to their corresponding tRNA. HARS2 ensures that the amino acid histidine is attached to the correct tRNA, guaranteeing its proper placement in the mitochondrial protein.

HARS2, a mitochondrial aminoacyl-tRNA synthetase, catalyzes the ATP-dependent attachment of histidine to the 3'-end of its specific tRNA molecule. This process involves the formation of an intermediate compound called His-AMP.

HARS2 is also known as HARSL, HARSR, HO3, HisRS, PRLTS2.

Associated Diseases


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