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- Description
- Phenotype
- Genes & alleles
- Genotyping
- Health & husbandry
- References
- Purchasing information
- Terms of Use
Description
The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.
Strain Information
Type Coisogenic; Mutant Strain; Spontaneous Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Species laboratory mouse Appearance
agouti
Related Genotype: A/AImportant Note
The first four litters born to homozygous females should be fostered. BALB/cByJ (Stock No. 001026) or B6EiD2F1/J (Stock No. 003550) are recommended as good foster mothers for this strain. This strain is homozygous for the retinal degeneration allele, Pde6brd1.Development
Toxic milk Jackson (Atp7btx-J) arose spontaneously in strain C3H/HeJ in 1988. One outcross was made to C3HeB/FeJ and then sibling matings were used to maintain the stock. The stock is maintained in the homozygous condition with the use of foster mothers.
Control Information
| Control | ||
|---|---|---|
| 000658 C3HeB/FeJ | ||
| Considerations for Choosing Controls | ||
Related Strains
Strains carrying Pde6brd1 allele
View Strains carrying Pde6brd1 (74 strains)
004297 B6.CXB1-Pde6brd10/J 005252 B6EiC3Sn.BLiA-Ts(1716)65Dn/DnJ 003647 B6EiC3Sn.BLiAF1 002802 C3.BLiA Pde6b+-Krd/J 001979 C3A.BLiA-Pde6b+.O20-Prph2Rd2/J 001912 C3A.BLiA-Pde6b+/J 003648 C3Sn.BLiA-Pde6b+/Dn 004766 C57BL/6J-Pde6brd1-2J/J 004828 FVB.129P2-Pde6b+ Tyrc-ch/AntJ 004808 STOCK Mapttm1(EGFP)Klt Tg(MAPT)8cPdav/J
Phenotype
Phenotype Information
Wilson Disease - Models with phenotypic similarity to human disease where etiologies involve orthologs.1
1 Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).
assigned by genotype
The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.
Atp7btx-J/Atp7btx-J
C3H/HeJ-Atp7btx-J/J
- lethality-postnatal
- postnatal lethality (MGI Ref ID J:150853)
- mice are moribund by 2 to 3 weeks of age
- however, mice fostered by wild-type dams exhibit increased survival
- liver/biliary system phenotype
- abnormal liver morphology (MGI Ref ID J:86701)
- the mitochondrial in the liver are abnormal at 3 months of age consisting of changes in the width of cristae with some cystic dilation at the tips
- liver cirrhosis (MGI Ref ID J:24233)
- mice exhibit cirrhosis of the liver that is reduced in subsequent generation with continuous fostering to wild-type dams
- in older homozygotes
- homeostasis/metabolism phenotype
- abnormal copper level (MGI Ref ID J:86701)
- copper levels in the brain are increased 1.2-fold at 4 months and 3-fold at 6 months
- nervous system phenotype
- abnormal brain morphology (MGI Ref ID J:24233)
- mice exhibit neuronal necrosis in the dorsal and lateral aspects of the frontal parietal and occipital lobes but not the temporal lobe
- abnormal cerebrum morphology (MGI Ref ID J:150853)
- at 1 week of age acute neuronal necrosis, histologically characterized by nuclear pyknosis with cytoplasmic shrinkage and eosinophilia, is found in the dorsal and lateral aspects of the frontal, parietal and occipital lobes, but not the temporal lobe, and the first and second layers of each section of the cortex tend not to be affected while the third to sixth layers are the most affected
- neuron degeneration (MGI Ref ID J:150853)
- pigmentation phenotype
- diluted coat color (MGI Ref ID J:24233)
- pale coat color
- touch/vibrissae phenotype
- curly vibrissae (MGI Ref ID J:150853)
- cellular phenotype
- necrosis (MGI Ref ID J:24233)
- mice exhibit neuronal necrosis in the dorsal and lateral aspects of the frontal parietal and occipital lobes but not the temporal lobe
- skin/coat/nails phenotype
- diluted coat color (MGI Ref ID J:24233)
- pale coat color
- other phenotype
- maternal effect (MGI Ref ID J:150853)
- the first litter is the most severely affected and the severity of the phenotype in pups decreases with each subsequent litter of the dam
- hematopoietic system phenotype
- increased eosinophil cell number (MGI Ref ID J:150853)
- in areas of neuronal necrosis
- immune system phenotype
- increased eosinophil cell number (MGI Ref ID J:150853)
- in areas of neuronal necrosis
This mouse can be used to support research in many areas including:Atp7btx-J related
Pde6brd1 relatedMetabolism Research
Mouse/Human Gene Homologs
Wilson disease
retinitis pigmentosa, autosomal recessive
Sensorineural Research
Retinal Degeneration
Genes & Alleles
Gene & Allele Information
| Allele Symbol | Atp7btx-J | ||
|---|---|---|---|
| Allele Name | toxic milk Jackson | ||
| Allele Type | Spontaneous | ||
| Strain of Origin | C3H/HeJ | ||
| Gene Symbol and Name | Atp7b, ATPase, Cu++ transporting, beta polypeptide | ||
| Chromosome | 8 | ||
| Gene Common Name(s) | Atp7a; Hts; PINA; PWD; WC1; WD; WND; Wilson protein; toxic milk; tx; | ||
| General Note | Tests at the University of Massachusetts proved its allelism to the original toxic milk mutation (J:28232). | ||
| Molecular Note | The mutation comprises a G to A base substitution at position 2135 in exon 8 of the gene. This is predicted to result in a Gly712Asp missense mutation in the second putative membrane-spanning domain of the encoded protein. [MGI Ref ID J:71896] | ||
| Allele Symbol | Pde6brd1 | ||
| Allele Name | retinal degeneration 1 | ||
| Allele Type | Spontaneous | ||
| Common Name(s) | Pdebrd1; rd; rd-1; rd1; rodless retina; | ||
Genotyping
Genotyping Information
Genotyping Protocols
Atp7btx-J, Pyrosequencing
Helpful Links
Genotyping resources and troubleshooting
References
References
Additional References
Atp7btx-J relatedBronson RT. 1995. Short papers: Acute cerebral neuronal necrosis in copper deficient offspring of female mice with the toxic milk mutation. Mouse Genome 93(1):152-154. [MGI Ref ID J:24233]
Bronson RT; Sweet HO; Davisson MT. 1995. Acute cerebral neuronal necrosis in copper deficient offspring of female mice with the toxic milk mutatiopn Mouse Genome 93(1):152-4. [MGI Ref ID J:150853]
Coronado V; Nanji M; Cox DW. 2001. The Jackson toxic milk mouse as a model for copper loading. Mamm Genome 12(10):793-5. [PubMed: 11668395] [MGI Ref ID J:71896]
Phinney AL; Drisaldi B; Schmidt SD; Lugowski S; Coronado V; Liang Y; Horne P; Yang J; Sekoulidis J; Coomaraswamy J; Chishti MA; Cox DW; Mathews PM; Nixon RA; Carlson GA; St George-Hyslop P; Westaway D. 2003. In vivo reduction of amyloid-beta by a mutant copper transporter. Proc Natl Acad Sci U S A 100(24):14193-8. [PubMed: 14617772] [MGI Ref ID J:86701]
Roberts EA; Lau CH; da Silveira TR; Yang S. 2007. Developmental expression of Commd1 in the liver of the Jackson toxic milk mouse. Biochem Biophys Res Commun 363(4):921-5. [PubMed: 17910951] [MGI Ref ID J:127349]
Roberts EA; Robinson BH; Yang S. 2008. Mitochondrial structure and function in the untreated Jackson toxic milk (tx-j) mouse, a model for Wilson disease. Mol Genet Metab 93(1):54-65. [PubMed: 17981064] [MGI Ref ID J:130273]
Sweet HO; Davisson MT. 1989. Toxic milk-J (tx<J>) Mouse News Lett 84:89. [MGI Ref ID J:28232]
Health & husbandry
The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.
Health & Colony Maintenance Information
Colony Maintenance
Breeding & Husbandry Litters must be fostered prior to 14 days of age. On an agouti background, pups are dirty gray in coat color. On a non-agouti background, pups are bronze/copper in color. The first 4 litters will have all pups phenotypically dirty white and dead by 14 days due to the reduced copper concentration in the birth mother's milk. Pups can be fostered at up to 10-14 days of age, and survive, even from the first litter. The fifth litter and beyond may be phenotypically normal in coat color and live normally to survive to a year of age. These healthy progeny are still homozygous and their first 1 to 4 litters will be affected. At The Jackson Laboratory BALB/cByJ or B6EiD2F1 are used as foster mothers for this colony.
Purchasing information
Pricing, Supply Level & Notes, Controls, General Terms & Conditions
Pricing
| Pricing for USA, Canada and Mexico shipping destinations |
|
Animals Provided
Price (US dollars $) Cryorecovery Fee $1900.00 At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.
Additional Supply Details
| Pricing for International shipping destinations |
|
Animals Provided
Price (US dollars $) Cryorecovery Fee $2470.00 At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.
Additional Supply Details
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Supply Details
| Standard Supply | Cryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information. |
|---|---|
| Supply Notes |
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| Important Note | |
| The first four litters born to homozygous females should be fostered. BALB/cByJ (Stock No. 001026) or B6EiD2F1/J (Stock No. 003550) are recommended as good foster mothers for this strain. This strain is homozygous for the retinal degeneration allele, Pde6brd1. | |
Control Information
| Control | ||
|---|---|---|
| 000658 C3HeB/FeJ | ||
| Considerations for Choosing Controls | ||
| USA, Canada and Mexico - Control Pricing Information for Genetically Engineered Mutant Strains. | ||
| International - Control Pricing Information for Genetically Engineered Mutant Strains. | ||
Payment Terms and Conditions
Terms are granted by individual review and stated on the customer invoice(s) and account statement. These transactions are payable in U.S. currency within the granted terms. Payment for services, products, shipping containers, and shipping costs that are rendered are expected within the payment terms indicated on the invoice or stated by contract. Invoices and account balances in arrears of stated terms may result in The Jackson Laboratory pursuing collection activities including but not limited to outside agencies and court filings.
See Terms of Use tab for General Terms and Conditions
The Jackson Laboratory's Genotype Promise
The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.Ordering and Purchasing Information
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JAX® Mice, Products & Services Conditions of Use
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