RPS6 phosphorylation status as a readout, Bcs1lp.S78G;mt-Cybp.D254N  livers had a tendency for increased mTORC1 activity, but this was unaffected by AOX (Fig. 7e), implying that mTORC1 was not a key player in the mt-ISR of CIII deficiency. This evidence concerns the gene RPS6 and hyperinsulinemic hypoglycemia, familial, 4.