Fig. 1 | Scientific Reports

Fig. 1

From: A robust high-throughput screening system to assess bacterial tyrosine ammonia lyase activity in the context of tyrosine inherited metabolic disorders

Fig. 1

The degradation pathway of tyrosine. At four steps of the pathway, tyrosine inherited metabolic disorders have been identified. Nitisinone, also known as NTBC, a substrate reduction therapy for hereditary tyrosinemia type 1 and alkaptonuria, inhibits the conversion of 4-hydroxyphenylpyruvate into homogentisate and thus decreases the production of the toxic metabolites in hereditary tyrosinemia type 1 and alkaptonuria2,3. However, tyrosine levels increase upon nitisinone administration, leaving patients at risk for ocular, cutaneous and neurological side effects2,3,4.

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