Fig. 4.

To recreate a milieu similar to the clinical situation of high protein diet, the medium was supplemented with branched-chain amino acids, isoleucine (5 mM) and valine (5 mM). Ammonia levels in supernatants of day 10 hepatocytes from the PA patient were significantly increased in supplemented media relative to culture in normal media (Fig. 4A) unlike healthy donor hepatocytes. Urea levels in the supernatants of day 10 PA patient hepatocytes were significantly decreased in the presence of supplemented media relative to normal media conditions (Fig. 4B) unlike healthy donor hepatocytes.