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. 2022 Dec 26;10(5):1759-1762.
doi: 10.1016/j.gendis.2022.11.013. eCollection 2023 Sep.

Short-term nitisinone discontinuation of hereditary tyrosinemia type 1 mice causes metabolic alterations in glutathione metabolism/biosynthesis and multiple amino acid degradation pathways

Colemonts-Vroninks Haaike  1   2 , Norman P Brendan  3 , Van Laere Sven  4 , Davison S Andrew  3   5 , Marcélis Lionel  6 , Casimir Georges  6 , Goyens Philippe  6 , Claes Paul  7 , De Bundel Dimitri  2 , Martens Geert  8   9 , Ranganath Lakshminarayan Rao  5 , Vanhaecke Tamara  7 , Gallagher James A  3 , De Kock Joery  1
Affiliations

Short-term nitisinone discontinuation of hereditary tyrosinemia type 1 mice causes metabolic alterations in glutathione metabolism/biosynthesis and multiple amino acid degradation pathways

Colemonts-Vroninks Haaike et al. Genes Dis. .
No abstract available

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Figures

Fig. 1
Figure 1
Transcriptional (by microarray) and metabolic (by LC-QTOF-MS) profiling of Fah-deficient mice upon seven-day nitisinone treatment discontinuation compared to continuously treated controls. (A) Summary of the experimental workflow including mouse gender, genotype and age, treatment groups, sample collection, and data analyses. (B) Ingenuity toxicological classification of transcriptional data using liver tissue and metabolic data using serum. The top x-axis and red line charts represent categories ranked by their -log (B–H P-value), the lower x-axis and bar charts represent the total number of differentially expressed molecules, and the black dashed line represents the B–H corrected P-value ≤0.05 threshold. (C) Summary of potentially affected metabolic pathways in serum samples, identified by high-resolution metabolomics. Pathways were visualized based on the KEGG pathway database (http://www.genome.jp/kegg/pathway.html). Identified metabolites that were more than 1.5-fold up- or down-regulated are colored in red and blue, respectively, and the metabolites in black were not identified or altered in this study. Metabolites with an asterisk (∗) are duplicates in this diagram to avoid the complexity of pathways. Solid and dashed arrows indicate respectively the single and multiple steps involved between two metabolites or pathways.
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References

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