Biogenesis of mitochondria. Defective assembly of the proteolipid into the mitochondrial adenosine triphosphatase complex in an oli2 mit- mutant of Saccharomyces cerevisiae.

A single mutation in the oli2 region of the mitochondrial DNA causes a charge alteration in a mitochondrially translated subunit of the mitochondrial ATPase (subunit 6; apparent Mr 20 000; apparent pI 6.9 and 7.1). This alteration leads to the defective assembly of the proteolipid subunit into the enzyme complex. The mutant, which is able to grow only very slowly by oxidative metabolism at 28 degrees C offers new possibilities for studying the assembly of the membrane sector (F0) into the mitochondrial ATPase complex and the role of subunit 6 in this process.