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Mitochondrial membrane potential and ATP production in primary disorders of ATP synthase. 2004

Alena Vojtísková, and Pavel Jesina, and Martin Kalous, and Vilma Kaplanová, and Josef Houstek, and Markéta Tesarová, and Daniela Fornůsková, and Jirí Zeman, and Audrey Dubot, and Catherine Godinot
Institute of Physiology and Centre for Integrated Genomics, Academy of Sciences of the Czech Republic, Prague, Czech Republic.

Studies of fibroblasts with primary defects in mitochondrial ATP synthase (ATPase) due to heteroplasmic mtDNA mutations in the ATP6 gene, affecting protonophoric function or synthesis of subunit a, show that at high mutation loads, mitochondrial membrane potential DeltaPsi(m) at state 4 is normal, but ADP-induced discharge of DeltaPsi(m) is impaired and ATP synthesis at state 3-ADP is decreased. Increased DeltaPsi(m) and low ATP synthesis is also found when the ATPase content is diminished by altered biogenesis of the enzyme complex. Irrespective of the different pathogenic mechanisms, elevated DeltaPsi(m) in primary ATPase disorders could increase mitochondrial production of reactive oxygen species and decrease energy provision.

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