Revisiting Quantification of Phenylalanine/Tyrosine Flux in the Ochronotic Pathway during Long-Term Nitisinone Treatment of Alkaptonuria

Changes in the phenylalanine (PHE)/tyrosine (TYR) pathway metabolites before and during homogentisic acid (HGA)-lowering by nitisinone in the Suitability of Nitisinone in Alkaptonuria (AKU) 2 (SONIA 2) study enabled the magnitude of the flux in the pathway to be examined. SONIA 2 was a 48-month rand...

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Bibliographic Details

Published in:	Metabolites
Main Authors:	Lakshminarayan R. Ranganath, Andrew T. Hughes, Andrew S. Davison, Milad Khedr, Richard Imrich, Mattias Rudebeck, Birgitta Olsson, Brendan P. Norman, George Bou-Gharios, James A. Gallagher, Anna M. Milan
Format:	Article
Language:	English
Published:	MDPI AG 2022-09-01
Subjects:	alkaptonuria pigment biliary excretion ochronosis nitisinone homogentisic acid
Online Access:	https://www.mdpi.com/2218-1989/12/10/920