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. Author manuscript; available in PMC: 2015 Sep 1.
Published in final edited form as: J Inherit Metab Dis. 2014 Feb 3;37(5):735–743. doi: 10.1007/s10545-013-9675-2

Figure 3. NTBC treatment reverses PKU-associated hypopigmentation.

Figure 3

Oral NTBC treatment for eight weeks yielded reversal of hypopigmentation in treated Pahenu2/enu2 mice (left) in comparison to untreated littermates (right). PKU-associated hypopigmentation is due to Phe-mediated competitive inhibition of tyrosinase enzyme, the rate-limiting step in melanin synthesis; increased Tyr and lower Phe due to NTBC treatment reverses tyrosinase inhibition.