- abnormal liver morphology / MGI
- hepatic necrosis / MGI
- abnormal kidney morphology / MGI
- hepatic steatosis / MGI
- ketoaciduria / MGI
- oxidative stress / MGI
- renal necrosis / MGI
- abnormal renal tubule epithelium morphology / MGI
- increased sensitivity to induced morbidity/mortality / MGI
- increased kidney apoptosis / MGI
129S/Sv-Gstz1tm1Jmfc/Cnbc
| Status | Available to order |
| EMMA ID | EM:04481 |
| Citation information | RRID:IMSR_EM:04481 Research Resource Identifiers (RRID) are persistent unique ID numbers assigned to help researchers cite key resources (e.g. antibodies, model organisms and software projects) in the biomedical literature to improve transparency and reproducibility in research. See https://www.rrids.org/ for more information. |
| International strain name | 129S/Sv-Gstz1tm1Jmfc/Cnbc |
| Alternative name | MAAI/GSTZ deficient |
| Strain type | Targeted Mutant Strains : Knock-out |
| Allele/Transgene symbol | Gstz1tm1Jmfc |
| Gene/Transgene symbol | Gstz1 |
Information from provider
| Provider | Jose Manuel Fernandez-Canon |
| Provider affiliation | Instituto de Biologia Malecular, Genomica y Proteomica, Universidad de leon |
| Genetic information | Removal of 60% of the Maai (Gstz1) gene (coding region). |
| Phenotypic information | Deficient in the MAAI/GSTZ enzyme (no enzymatic activity), involved in the catabolic pathway of phenylalanine/tyrosine. Sensitive to high phenylalanine diet. Accumulates maleylacetoacetate (MAA) and fumarylacetoacetate (FAA), which are toxic compounds. |
| Breeding history | Mutant mice were generated in 2001. They have been backcrossed with 129/SvPas (Charles River Lab) in 2003 to avoid strain degeneration. |
| References |
|
| Homozygous fertile | yes |
| Homozygous viable | yes |
| Homozygous matings required | no |
| Immunocompromised | no |
Information from EMMA
| Archiving centre | CNB-CSIC, Centro Nacional de Biotecnologia, Madrid, Spain |
| Animals used for archiving | heterozygous 129S2/SvPas males, wild-type 129S2/SvPas females |
| Stage of embryos | 8-cell |
Disease and phenotype information
MGI phenotypes (allele matching)
MGI phenotypes (gene matching)
- decreased leukocyte cell number / MGI
- cellular necrosis / MGI
- abnormal liver morphology / MGI
- abnormal hepatocyte morphology / MGI
- decreased body weight / MGI
- weight loss / MGI
- hepatic necrosis / MGI
- liver inflammation / MGI
- premature death / MGI
- abnormal kidney morphology / MGI
- chronic inflammation / MGI
- hepatic steatosis / MGI
- dystrophic cardiac calcinosis / MGI
- ketoaciduria / MGI
- increased liver weight / MGI
- oxidative stress / MGI
- increased kidney weight / MGI
- renal necrosis / MGI
- decreased spleen weight / MGI
- abnormal renal tubule epithelium morphology / MGI
- increased sensitivity to induced morbidity/mortality / MGI
- increased kidney apoptosis / MGI
Literature references
- Maleylacetoacetate isomerase (MAAI/GSTZ)-deficient mice reveal a glutathione-dependent nonenzymatic bypass in tyrosine catabolism.;Fernández-Cañón José Manuel, Baetscher Manfred W, Finegold Milton, Burlingame Terry, Gibson K Michael, Grompe Markus, ;2002;Molecular and cellular biology;22;4943-51; 12052898
- GSTZ1 sensitizes hepatocellular carcinoma cells to sorafenib-induced ferroptosis via inhibition of NRF2/GPX4 axis.;Wang Qiujie, Bin Cheng, Xue Qiang, Gao Qingzhu, Huang Ailong, Wang Kai, Tang Ni, ;2021;Cell death & disease;12;426; 33931597
- GSTZ1-1 Deficiency Activates NRF2/IGF1R Axis in HCC via Accumulation of Oncometabolite Succinylacetone.;Yang Fan, Li Jingjing, Deng Haijun, Wang Yihao, Lei Chong, Wang Qiujie, Xiang Jin, Liang Li, Xia Jie, Pan Xuanming, Li Xiaosong, Long Quanxin, Chang Lei, Xu Ping, Huang Ailong, Wang Kai, Tang Ni, ;2019;The EMBO journal;38;e101964; 31267557
- GSTZ1 deficiency promotes hepatocellular carcinoma proliferation via activation of the KEAP1/NRF2 pathway.;Li Jingjing, Wang Qiujie, Yang Yi, Lei Chong, Yang Fan, Liang Li, Chen Chang, Xia Jie, Wang Kai, Tang Ni, ;2019;Journal of experimental & clinical cancer research : CR;38;438; 31666108
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