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This week, 15-21 September 2019, is Mitochondrial Disease Awareness Week.

Around the world Global Mitochondrial Disease Awareness Week (GMDAW) will be marked with educational, fundraising and advocacy efforts designed to raise awareness about mitochondrial disease (mito).

The MBU's website will follow a green theme for the duration of this week.

Stochastic mutations in the mitochondrial genome (mtDNA) have been linked to many diseases, and their accumulation has been proposed to act as a driving force in the ageing process itself. For the past 15 years the main animal model used to study this phenomenon has been the ‘mutator’ mouse, harbouring a proofreading defective mtDNA polymerase (POLG). These models accumulate high levels of mtDNA point mutations, but also large and small deletions and mtDNA depletion, causing progeroid (accelerated ageing) phenotypes.

Adenosine triphosphate (ATP), the fuel of life, is produced by a molecular machine consisting of two motors linked by a rotor. One generates rotation by consuming energy derived from oxidative metabolism or photosynthesis; the other uses energy transmitted by the rotor to make ATP from adenosine diphosphate and phosphate. In many species, the machine is reversible, and various mechanisms regulate reversal.