ATP14 encodes subunit h of yeast mitochondrial ATP synthase (1). The ATP synthase complex utilizes proton motive force to generate ATP from ADP and P_i (3). The structure of this enzyme complex is highly conserved among diverse organisms and consists of two major components, soluble F₁ and membrane-bound F₀, each of which contains many subunits. Subunit h is one of only a few known ATP synthase subunits that are unique to yeast mitochondria; no subunit h homologs have been identified in either mammalian mitochondria or bacteria. Although the specific function of this protein is not yet known, it is essential for F₀ assembly and ATP synthase activity (4 and references therein). Its N-terminal 32 amino acids are removed during import into mitochondria (5). Deletion of ATP14, like deletions in many genes necessary for the function or maintenance of mitochondria, leads to a "petite" phenotyepe that is slow-growing and unable to survive on nonfermentable carbon sources (1).

General ATP synthase structure and function are reviewed in references 3 and 6. For a review that is specific to yeast, see reference 4.

Last updated: 2007-10-23