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ATP5G3 belongs to the ATPase C chain family. Mitochondrial membrane ATP synthase (F1F0 ATP synthase or Complex V) produces ATP from ADP in the presence of a proton gradient across the membrane which is generated by electron transport complexes of the respiratory chain. ATP5G3 is the major protein stored in the storage bodies of animals or humans affected with ceroid lipofuscinosis (Batten disease).