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The photomacrographs were analyzed using ImageJ software (Wayne Rasband, MA, United States) to assess ulceration area according to Szabo and Hollander (Szabo and Hollander, 1989)

Priebe A, Tan L, Wahl H, Kueck A, He G, Kwok R et al

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Disclosures: Alyssa Goldberg: Nothing to Disclose, William Hollenbach: Nothing to Disclose, Cara Mack: Nothing to Disclose, Beth Tamburini: Nothing to Disclose 2499 REQUIRED DOSE AND EFFECTS OF NTBC TREATMENT FOR HEREDITARY TYROSINEMIA TYPE 1: CONFIRMATIONS AND SURPRISES FROM A NOVEL, REPRESENTATIVE HUMAN MODEL Farzaneh Tamnanloo 1 Quang Toan Pham 1 Marie-Agns MCallum 1 Denis Cyr 2 Paula Waters 2 Emilie Beaulieu 1 Yannick Doyon 3 Ugur Halac 4 Claudia Raggi 1 Massimiliano Paganelli 4 , 1 Liver Tissue Engineering and Cell Therapy Laboratory, CHU Sainte-Justine, Montral, Canada, 2 Department of Biochemistry, Universit de Sherbrooke, Sherbrooke, Canada, 3 Department of Molecular Medicine, Universit Laval, Quebec, Canada, 4 Department of Pediatrics, Universit de Montral, Montral, Canada Background: Hereditary Tyrosinemia Type 1 (HT1) is a severe genetic liver disorder caused by a deficiency in fumarylacetoacetate hydrolase (FAH), a key enzyme of tyrosine metabolic pathway