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. 2015 Oct;4(4):290–303. doi: 10.3978/j.issn.2224-4336.2015.10.08

Figure 1.

Figure 1

Schematic of the tyrosine degradation pathway. FAH deficiency in hereditary tyrosinemia type I (HT1) leads to accumulation of fumarylacetoacetate and, maleylacetoacetate, with production of succinylacetone (SA) that becomes detectable in the urine and plasma. NTBC (nitisinone) is a potent inhibitor of the 4-HPPD enzyme; (B, C) photomicrograph of liver from an 8-month old patient with HT1 showing typical micro- and macro-nodular cirrhosis (B, magnification, 50×) and a dysplastic nodule (C, magnification, 200×). TAT, tyrosine aminotransferase; 4-HPPD, 4-OH phenylpyruvate dioxygenase; HGD, homogentisate dioxygenase; MAI, maleylacetoacetate isomerase; FAH, fumarylacetoacetate hydrolase.