Adenine nucleotide translocases (ANTs) are crucial for mitochondrial integrity and bioenergetic metabolism. There are four isoforms of ANTs (ANT1-4). Under physiological conditions, ANTs primarily engage in the exchange of adenosine diphosphate (ADP) and adenosine triphosphate (ATP) across mitochondrial membranes. They are also potentially significant components of the mitochondrial permeability transition pore (mPTP), contributing to processes such as cellular apoptosis and proton leakage. Impairment of ANTs can lead to mitochondrial dysfunction, which holds significant pathological implications in metabolic diseases, cardiomyopathies, and cancer progression. This review summarizes recent advancements and knowledge regarding ANTs, aiming to offer new insights into potential therapeutic strategies targeting ANTs in diseases.