Basic Information
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Description
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- Fah is the final enzyme in the tyrosine metabolism pathway, primarily expressed in the liver and kidneys. Deficiency of FAH is associated with Type 1 hereditary tyrosinemia (HT). As a result of the severe liver damage induced by Fah knockout (KO), this mouse model allows for the reconstruction of a functional human liver utilizing primary human hepatocytes.
- Gene editing strategy: The exons 2-14 of mouse Fah gene were knocked out in B-NDG Fah KO mice.
- mRNA expression analysis: Mouse Fah mRNA were detectable only in B-NDG mice but not in homozygous B-NDG Fah KO mice.
- Protein expression analysis: Fah was only detected in liver and kidney of B-NDG mice but not in homozygous B-NDG Fah KO mice.
- Application: This product is used for human hepatocytes transplantation and for evaluating the efficacy and safety of drugs associated with liver diseases.
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Targeting strategy
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Gene targeting strategy for B-NAG Fah KO mice. The exons 2-14 of mouse Fah gene were knocked out in B-NDG Fah KO mice.
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mRNA expression analysis in B-NDG Fah KO mice
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Strain specific analysis of Fah mRNA expression in B-NDG mice and B-NDG Fah KO mice by RT-PCR. Liver RNA was isolated from B-NDG mice (+/+) and homozygous B-NDG Fah KO mice (-/-). cDNA libraries were synthesized by reverse transcription, followed by PCR with mouse Fah primers. Mouse Fah mRNA was detectable only in B-NDG mice but not in homozygous B-NDG Fah KO mice.
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Protein expression analysis in B-NDG Fah KO mice
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Western blot analysis of Fah protein expression in homozygous B-NDG Fah KO mice. Various tissue lysates were collected from B-NDG mice (+/+) and homozygous B-NDG Fah KO mice (-/-), and then analyzed by western blot with anti-Fah antibody (proteintech, 14928-1-AP). A total of 40 μg of protein was loaded for western blotting analysis. Fah was only detected in liver and kidney of B-NDG mice.