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| Status |
Public on Dec 23, 2020 |
| Title |
Gene expression profiling of Fah-deficient mouse livers upon short-term nitisinone discontinuation |
| Organism |
Mus musculus |
| Experiment type |
Expression profiling by array
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| Summary |
Hereditary tyrosinemia type 1 (HT1) is a severe genetic disorder that affects the liver due to a defective fumarylacetoacetate hydrolase (Fah) enzyme in hepatocytes. The drug nitisinone (NTBC) has offered a life-saving treatment for HT1 patients by inhibiting the upstream enzyme 4-hydroxyphenylpyruvate dioxygenase (HPD).
We used microarray analyses to define the impact of short-term (ie. seven days) NTBC therapy discontinuation on the gene expression profile of liver tissue of Fah-deficient mice. Consequently, we investigated the modulation of canonical pathways related to oxidative stress, glutathione metabolism and liver regeneration.
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| Overall design |
FRG mice (C57Bl/6J background) are characterized by a triple knockout of Fah−/−, Rag2−/− and Il2rg−/− and served as experimental model for HT1. FRG mice (in triplicate) were either continuously treated with 16 mg/l NTBC through their drinking water or deprived from NTBC therapy for seven consecutive days. Withdrawal samples were collected seven days after NTBC discontinuation.
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| Contributor(s) |
Colemonts-Vroninks H, Neuckermans J, Claes P, Branson S, Martens GA, Vanhaecke T, De Kock J |
| Citation(s) |
33375092 |
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| Submission date |
Nov 13, 2020 |
| Last update date |
Jan 26, 2021 |
| Contact name |
Joery De Kock |
| E-mail(s) |
jdekock@vub.ac.be
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| Phone |
+32 2 477 45 17
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| Organization name |
Vrije Universiteit Brussel (VUB)
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| Department |
In Vitro Toxicology and Dermato-Cosmetology
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| Street address |
Laarbeeklaan 103
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| City |
Brussels |
| ZIP/Postal code |
1090 |
| Country |
Belgium |
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| Platforms (1) |
| GPL1261 |
[Mouse430_2] Affymetrix Mouse Genome 430 2.0 Array |
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| Samples (6)
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| GSM4908529 |
FRG seven days withdrawal condition, biological rep1 |
| GSM4908530 |
FRG seven days withdrawal condition, biological rep2 |
| GSM4908531 |
FRG seven days withdrawal condition, biological rep3 |
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| Relations |
| BioProject |
PRJNA678367 |
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