John.Harrison at mailbox.uq.oz.au wrote:
>> This causes Lebers hereditary optic neuropathy.
> What is the best way of "boosting" energy production via mitochondria
> with this mutation present in 100% of cells.
As the mutation at 11778 affects the ND4 gene of Complex I, and
results in a decrease in oxidative phosphorylation, it follows that
any treatment able to increase either the efficiency of oxidative
phosphorylation or its rate in the affected cells should be able to
delay the onset of the disease. It is possible to accomplish this
in spite of the defect in Complex I.
If we consider that the symptoms caused by the 11778 mutation are
essentially due to a Complex I defect (ignoring environmental
factors), and that ubiquinone is the rate-limiting compound of the
activity of complex I, then increasing the concentration of
ubiquinone within the mitochondrial inner membrane will cause
an increase in the production of ATP. Addition of ubiquinone to
the system should enhance the respiratory rate above the current
physiological (diseased) state.
The only problem with this treatment theoretically, is to
determine whether exogenous administration of ubiquinone is
effective in raising the concentration of ubiquinone in the
mitochondria.
Best Regards,
--
Asha Pharma
http://www.nethomes.com/asha
