ATP Synthase Mitochondrial F1 Complex Assembly Factor 2 (ATPAF2)

ATPAF2 encodes an assembly factor for the F(1) component of the mitochondrial ATP synthase. This protein binds specifically to the F1 alpha subunit and is thought to prevent this subunit from forming nonproductive homooligomers during enzyme assembly. This gene is located within the Smith-Magenis syndrome region on chromosome 17. An alternatively spliced transcript variant has been described, but its biological validity has not been determined.he deduced 289-amino acid protein contains an N-terminal mitochondrial targeting sequence. EST database analysis and SAGE data indicated that ATPAF2 is widely expressed at moderate abundance.ATPAF2 interacted strongly with the ATP synthase F1 alpha subunit (ATP5A1) and weakly with the F1 beta subunit (ATP5B).