C57BL/6JCya-Fahem1flox/Cya
Common Name:
Fah-flox
Product ID:
S-CKO-02349
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
Price:
Contact for Pricing
Basic Information
Strain Name
Fah-flox
Strain ID
CKOCMP-14085-Fah-B6J-VA
Gene Name
Product ID
S-CKO-02349
Gene Alias
swst
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
7
Phenotype
Document
Application
--
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Fahem1flox/Cya mice (Catalog S-CKO-02349) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000032865
NCBI RefSeq
NM_010176
Target Region
Exon 3~4
Size of Effective Region
~1.1 kb
Detailed Document
Overview of Gene Research
Fah, short for fumarylacetoacetate hydrolase, is the last enzyme in the tyrosine catabolic pathway. It belongs to the FAH superfamily of enzymes, whose members share conserved regions forming the FAH-domain and catalyze various reactions. In both prokaryotes and eukaryotes, these enzymes are essential for degrading aromatic compounds. In prokaryotes, they help generate energy and useful metabolites from complex carbon sources, while in eukaryotes, they may have acquired regulatory functions beyond catabolism [2].
Several animal models of Fah deficiency have been developed, such as mice, pigs, and rats. Fah-positive hepatocytes have a selective growth advantage in mutant livers, enabling extensive repopulation of the diseased organ. Thus, Fah-knockout mice have become crucial for liver biology research, including studies on liver stem cells and hepatic gene therapy. Immune-deficient Fah-knockout mice can be repopulated with human hepatocytes, creating “mice with human livers” which serve as important preclinical models for infectious diseases, metabolism, and gene therapy. Additionally, Fah-knockout mouse models have also been used to study BEC-to-hepatocyte conversion and sleep-wake pattern alterations related to chronic tyrosinemia [1,3,4].
In conclusion, Fah is essential for tyrosine catabolism and has implications in multiple biological processes. Fah-knockout mouse models have significantly contributed to research in liver-related diseases, infectious diseases, metabolism, and the understanding of BEC-to-hepatocyte conversion and sleep-wake pattern regulation. These models provide valuable insights into the role of Fah in health and disease, facilitating further exploration of potential therapeutic strategies.
References:
1. Grompe, Markus. . Fah Knockout Animals as Models for Therapeutic Liver Repopulation. In Advances in experimental medicine and biology, 959, 215-230. doi:10.1007/978-3-319-55780-9_20. https://pubmed.ncbi.nlm.nih.gov/28755199/
2. Weiss, Alexander K H, Loeffler, Johannes R, Liedl, Klaus R, Gstach, Hubert, Jansen-Dürr, Pidder. 2018. The fumarylacetoacetate hydrolase (FAH) superfamily of enzymes: multifunctional enzymes from microbes to mitochondria. In Biochemical Society transactions, 46, 295-309. doi:10.1042/BST20170518. https://pubmed.ncbi.nlm.nih.gov/29487229/
3. Wang, Xingrui, Pu, Wenjuan, Zhu, Huan, Zhang, Mingjun, Zhou, Bin. . Establishment of a Fah-LSL mouse model to study BEC-to-hepatocyte conversion. In Biophysics reports, 9, 309-324. doi:10.52601/bpr.2023.230034. https://pubmed.ncbi.nlm.nih.gov/38524699/
4. Yang, Shuzhang, Siepka, Sandra M, Cox, Kimberly H, Tu, Benjamin, Takahashi, Joseph S. 2019. Tissue-specific FAH deficiency alters sleep-wake patterns and results in chronic tyrosinemia in mice. In Proceedings of the National Academy of Sciences of the United States of America, 116, 22229-22236. doi:10.1073/pnas.1904485116. https://pubmed.ncbi.nlm.nih.gov/31611405/
Quality Control Standard
Sperm Test
Pre-cryopreservation: Measurement of sperm concentration, determination of sperm viability.
Post-cryopreservation: A vial of cryopreserved sperms is selected for in-vitro fertilization from each batch.
Environmental Standards:SPF
Available Region:Global
Source:Cyagen