Aminoacyl-tRNA synthetases are a class of enzymes that catalyze the attachment of specific amino acids to their corresponding tRNA molecules. They are divided into two major families, comprising over 20 members, with each member corresponding to a specific amino acid. These enzymes consist of multiple structural domains and are found in the cytoplasm and mitochondria. They play a crucial role in protein biosynthesis by ensuring the correct recognition and attachment of amino acids to tRNA, thereby participating in the translation process. The gene expression of these enzymes is cell type-specific and tightly regulated. Mutations may lead to errors in amino acid recognition and protein synthesis, associated with various genetic diseases.