| Modification Notes |
The mitochondrial ATP synthases transduce the energy contained in the membrane’s electrochemical proton gradients into the energy required for synthesis of high-energy phosphate bonds. F1 is the hydrophilic domain of ATPase that has three identical alpha subunits, three identical beta subunits and three additional subunits. Each ATPase contains three catalytic sites for synthesis, with one site located in each of the three beta subunits. ATPAF1 (ATP synthase mitochondrial F1 complex assembly factor 1), also known as its yeast homolog Atp11p, is a 328 amino acid mitochondrial protein that is required for the assembly of F1-beta and F1-alpha subunits into the mitochondrial ATPase. Both ATPAF1 and ATPAF2 are broadly conserved in eukaryotes and are widely expressed, suggesting that they are essential housekeeping proteins. Due to their influence on enzyme assembly, it has been suggested that evaluation of ATPAF1 and ATPAF2 may be of interest in patients with ATP synthase deficiencies in which the underlying biochemical defect is unknown. |