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To determine CI activity (NADH-dependent OCR), MAS plus alamethicin (2.5 g ml 1 ) and cytochrome c (10 g ml 1 ) was added onto the cells followed by 5 mM pyruvate + 5 mM malate + 1 mM NADH + 10 nM recombinant perfringolysin O at port A, 2 M rotenone + 4 M antimycin A at port B, 0.5 mM TMPD + 1 mM ascorbic acid at port C and 50 mM azide at port D

Additional symptoms often present during an episode including dizziness, paleness of the skin (pallor), lack of energy (listlessness) and, abdominal pain and headaches

It is possible therefore that a poor gut biome, or indeed even the source of ALCAR/LC may be important in describing outcomes

5 .