Alanine--TRNA Ligase, Mitochondrial (AARS2) Antibody

Background

AARS2 is an essential mitochondrial enzyme responsible for catalyzing the ligation of alanine to its corresponding tRNA (tRNA^Ala) during mitochondrial protein synthesis. This process is crucial for the translation of mitochondrial-encoded proteins, which are integral components of the oxidative phosphorylation system. Structurally, AARS2 contains a catalytic domain that ensures high specificity for alanine and tRNA^Ala, contributing to the accuracy of mitochondrial protein translation. AARS2 is predominantly expressed in energy-demanding tissues such as the brain, heart, and skeletal muscle, underscoring its role in maintaining mitochondrial function. Mutations in the AARS2 gene have been linked to mitochondrial diseases, including leukodystrophy and cardiomyopathy, which are characterized by impaired mitochondrial protein synthesis and energy production. These conditions highlight the critical importance of AARS2 in cellular energy metabolism and mitochondrial integrity. Emerging research also suggests potential links between AARS2 dysfunction and broader neurodegenerative disorders, making it a significant target for therapeutic exploration.