This is in agreement with the data showing that in hyperammonemia, the steady-state intramitochondrial ammonia concentration depends on many factors [43,93], especially on the rate of its transport from the blood to mitochondria; this relies particularly on the availability of mitochondrial aquaporin-8, a membrane channel, permeable to ammonia [94,95], on ammonia-forming endogenous reactions in mitochondria [66,67] and glutamine synthetase activity [96]. Here, GLUL is linked to Hyperammonemia.