Predicted to enable ADP binding activity and ATP binding activity. Predicted to contribute to proton-transporting ATP synthase activity, rotational mechanism. Involved in electron transport chain and lipid storage. Located in mitochondrion. Is expressed in adult head; adult heart; adult hemolymph; germline cell; and wing disc. Human ortholog(s) of this gene implicated in combined oxidative phosphorylation deficiency 22; mitochondrial complex V (ATP synthase) deficiency nuclear type 4A; and mitochondrial complex V (ATP synthase) deficiency nuclear type 4B. Orthologous to human ATP5F1A (ATP synthase F1 subunit alpha).
FB Description
bellwether (blw) encodes the alpha subunit of the mitochondrial F1F0 ATP synthase complex (complex V), the final enzyme of the oxidative phosphorylation pathway. Complex V catalyzes the synthesis of ATP from ADP and inorganic phosphate using the electrochemical proton gradient across the inner mitochondrial membrane.