ABHD18 (α/β hydrolase domain-containing protein 18) is a transmembrane protein in mammals whose function has not yet been fully elucidated. It belongs to the large α/β hydrolase superfamily, whose members typically possess hydrolase activity and are involved in a variety of metabolic reactions. The ABHD18 protein is localized on the lysosomal membrane and may participate in intracellular lipid metabolism or signal transduction processes. Recent studies have shown that ABHD18 is the key enzyme in human cells that catalyzes the deacylation of cardiolipin to MLCL. The loss of ABHD18 function can significantly alleviate mitochondrial supercomplex defects, energy metabolism disorders, and cardiomyopathy phenotypes caused by TAZ deficiency, even achieving a "genetic suppression" effect in mice and patient cells. This not only reveals ABHD18 as a core node in the cardiolipin remodeling pathway but also provides an entirely new therapeutic approach for mitochondrial diseases such as Barth syndrome.