ATP synthase mitochondrial F1 complex assembly factor 2 (ATPAF2)

ATP synthase mitochondrial F1 complex assembly factor 2 (ATPAF2) is a protein required for the assembly of the F1 component of the mitochondrial ATP synthase, an essential enzyme complex that produces ATP in mitochondria. ATPAF2 specifically binds to the F1 alpha subunit, where it is thought to prevent this subunit from forming nonproductive homooligomers during assembly, thereby ensuring correct complex formation[1][5][6][7]. Mutations in ATPAF2 can lead to diseases such as mitochondrial complex V deficiency, and the gene is located within the Smith-Magenis syndrome critical region[1]. ATPAF2 is not a classical druggable target (such as a receptor, enzyme, or transporter) but is a chaperone-like assembly factor, making it functionally distinct from canonical pharmacological targets[1][6].

Other names

ATP12ATP12pAtp12pLP3663MGC29736MC5DN1ATPAF2ATP12 homolog