129S/Sv-Gstz1tm1Jmfc/Cnbc

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EMMA IDEM:04481
Citation informationRRID:IMSR_EM:04481 

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International strain name129S/Sv-Gstz1tm1Jmfc/Cnbc
Alternative nameMAAI/GSTZ deficient
Strain typeTargeted Mutant Strains : Knock-out
Allele/Transgene symbolGstz1tm1Jmfc
Gene/Transgene symbolGstz1

Information from provider

ProviderJose Manuel Fernandez-Canon
Provider affiliationInstituto de Biologia Malecular, Genomica y Proteomica, Universidad de leon
Genetic informationRemoval of 60% of the Maai (Gstz1) gene (coding region).
Phenotypic informationDeficient 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 historyMutant mice were generated in 2001. They have been backcrossed with 129/SvPas (Charles River Lab) in 2003 to avoid strain degeneration.
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
Homozygous fertileyes
Homozygous viableyes
Homozygous matings requiredno
Immunocompromisedno

Information from EMMA

Archiving centreCNB-CSIC, Centro Nacional de Biotecnologia, Madrid, Spain
Animals used for archivingheterozygous 129S2/SvPas males, wild-type 129S2/SvPas females
Stage of embryos8-cell

Disease and phenotype information

MGI phenotypes (allele matching)
  • 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
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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Requesting frozen sperm or embryos is generally advisable wherever possible, in order to minimise the shipment of live mice.

  • Frozen embryos. Delivered in 4 weeks (after paperwork in place). €1740*
  • Frozen sperm. Delivered in 4 weeks (after paperwork in place). €1740*
  • Rederivation of mice from frozen stock, delivery time available upon request . €3880*

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Practical information

Genotyping protocol

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Legally binding conditions for the transfer

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