Journal
BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS
Volume 1757, Issue 9-10, Pages 1400-1405Publisher
ELSEVIER SCIENCE BV
DOI: 10.1016/j.bbabio.2006.04.006
Keywords
mitochondrial diseases; ATP synthase; biogenesis; ATP6; ATP12; energy provision; reactive oxygen species
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ATP synthase is a key enzyme of mitochondrial energy conversion. In mammals, it produces most of cellular ATP. Alteration of ATP synthase biogenesis may cause two types of isolated defects: qualitative when the enzyme is structurally modified and does not function properly, and quantitative when it is present in insufficient amounts. In both cases the cellular energy provision is impaired, and diminished use of mitochondrial Delta mu H+ promotes ROS production by the mitochondrial respiratory chain. The primary genetic defects have so far been localized in mtNA ATP6 gene and nuclear ATP12 gene, however, involvement of other nuclear genes is highly probable. (c) 2006 Elsevier B.V. All rights reserved.
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