Mouse Strain information
APB ID 1952
Added 03/09/2007
Last Edited 24/08/2015
Strain name: BALB/c-Gstz1tm1Pgb/AnuApb
Nickname: BALB/c.Zeta
Genetics
Strain Types Mutant strain
Genetic Details Targeted knockout
Mode of Inheritance Recessive
1.  Affected Gene Name: glutathione transferase zeta 1 (maleylacetoacetate isomerase)  
Chromosome 12
Affected Gene Symbol Gstz1
Protein expression of altered gene: Nil
Genetic alteration of gene: Exons 6-8 were replaced with a neomycin resistance gene. PCR and Southern blot confirmed recombination in ES cells and Northern blot and Western blot indicated lack of transcript and protein in mutant organs.
Location on chromosome (bp): 88488668-88505671 bp, + strand (From Ensembl annotation of NCBI Build 37)
MGI Gene Accession ID

Please also use this link to determine if other mutants have been registered with MGI (Mouse Genome Informatics)

MGI:1341859
Synonyms maleylacetoacetate isomerase
Allele Name glutathione transferase zeta 1 (maleylacetoacetate isomerase); targeted mutation 1, Philip G Board
Allele Symbol Gstz1tm1Pgb
MGI Allele Accession ID MGI:3050206
Mutant Construction Technique None
Phenotype
Phenotype Homozygous State Increased sensitivity to induced morbidity/mortality: 3% phenylalanine in the water is lethal to young homozygotes, less than 28 days old, but not to older homozygotes, greater than 66 days old. Premature death: several mutants died after 1 year of age from heart pathologies. Dystrophic cardiac calcinosis: * in some mutants over 1 year of age calcification of the myocardium in the right atrium and/or ventricle is seen * calcification is also seen in 3 of 8 young mutants that died after 3% phenylalanine exposure. Cellular necrosis: * in some mutants over 1 year of age myocyte necrosis is seen * myocyte necrosis is also seen in 2 of 8 young mutants that died after 3% phenylalanine exposure. Decreased body weight: male homozygotes weigh slightly less than wild-type or heterozygous males. Weight loss: 3% phenylalanine in the water results in greater weight loss and growth restriction in male homozygotes compared to wild-type mice. Decreased leukocyte cell number: 3% phenylalanine in the water results in decreased total white cell counts in mutants but not in wild-type mice. Decreased spleen weight: * spleen weight is significantly decreased in female homozygotes and tends to be decreased in male homozygotes compared to wild-type mice. * 3% phenylalanine in the water results in a dramatic decrease in spleen weight in homozygotes with reduction in both white and red pulp, but an increase in spleen weight in wild-type mice. Chronic inflammation: in some mutants over 1 year of age chronic inflammation is seen in the heart. Liver inflammation: at 6 weeks and 6 months of age multifocal hepatitis is seen. Abnormal hepatocyte morphology: large pleomorphic mitochondria are seen in hepatocytes from mutants. Increased liver weight: on a normal diet liver weight is greater in homozygotes compared to wild-type mice. Hepatic necrosis: * at 6 months of age 3 of 7 homozygotes show hepatocyte necrosis. * 3 out of 8 homozygotes that died after 3% phenylalanine exposure displayed hepatocyte necrosis. Hepatic steatosis: 5 out of 8 homozygotes that died after 3% phenylalanine exposure displayed macrovesicular steatosis. Increased kidney weight: * kidney weight is greater in homozygotes compared to wild-type mice. * 3% phenylalanine in the water results in an increase in kidney weight in homozygotes but not in wild-type mice.
Original Genetic Background BALB/c
Genetic Background Currently Maintained BALB/c
Strain identification
How is this strain characterised? Genotyping
Fertility and Strain maintenance
Fertility and Strain maintenance
Relevant bibliographic / database references
Strain Specific References
Other Related References
General information
Associated IP rights? No
Does it Model a Human Condition? Yes
Applicable Research Areas Models for human disease
Cell biology
Metabolism
Developmental biology
Keywords
  • GST
  • maleylacetoacetate, maleylacetone (MA) and succinylacetone
  • phenylalanine and tyrosine
  • Keap1/Nrf2 pathway
  • oxidative stress
  • inflammation
  • glutathione transferase
Is the strain available in any form to other researchers Yes
APB stock stored as: Cryopreserved sperm (from 5 mice)

APB stock genotyped verified by: Unverified
Are live mice available? No
MTA needs to be signed? Yes


   

 


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