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. Author manuscript; available in PMC: 2012 Nov 1.
Published in final edited form as: Mol Genet Metab. 2011 Aug 4;104(3):235–240. doi: 10.1016/j.ymgme.2011.07.027

Figure 2. Physiologic model.

Figure 2

Phenylalanine clearance may be limited either by the rate of phenylalanine transport into PAH-expressing hepatocytes or by total PAH activity in liver. If phenylalanine clearance is primarily limited by PAH activity, then fewer wild-type hepatocytes with 100% PAH activity will be required to correct hyperphenylalaninemia than Pahenu2/+ hepatocytes with only partial PAH activity. If phenylalanine clearance is primarily related to transmembrane phenylalanine transport, then wild-type and Pahenu2/+ hepatocytes would be equally effective in restoring phenylalanine clearance.