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Strain Name:

C3HeB/FeJ-Atp7btx-J/J

Stock Number:

001576

Availability:

Repository-Cryopreserved

Price and Supply Information

General Terms and Conditions

Genes & Alleles   Atp7b;   Atp7btx-J;   Pde6b;   Pde6brd1;

Product Information

Strain Details

Type JAX® GEMM® Strain - Coisogenic
Additional information on JAX® GEMM® Strains.
Type JAX® GEMM® Strain - Mutant Strain
Type JAX® GEMM® Strain - Spontaneous Mutation
Specieslaboratory mouse

Appearance
agouti
Related Genotype: A/A

Important Note
The first 4 litters born to homozygous females should be fostered. BALB/cByJ (Stock No. 001026) or B6EiD2F1 (Stock No. 003550) are recommended as good foster mothers for this strain. This strain is homozygous for the retinal degeneration allele, Pde6brd1.

Strain 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.

Related Disease (OMIM) Terms

Wilson Disease
Mammalian Phenotype Terms 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:24233)
    • 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
  • 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
  • pigmentation phenotype
  • diluted coat color (MGI Ref ID J:24233)
  • touch/vibrissae phenotype
  • curly vibrissae (MGI Ref ID J:24233)
  • 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)

Gene & Allele Details

Allele Symbol Atp7btx-J
Allele Name toxic milk Jackson
Strain of OriginC3H/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
Common Name(s) rd; rd-1; rd1; rodless retina;

Control Information

  Allele   Control
 Atp7btx-J  000658 C3HeB/FeJ
 
  Considerations for Choosing Controls
  Control Pricing Information for JAX® GEMM® Strains

Colony Maintenance

Breeding & HusbandryLitters 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.

Related Strains

Strains carrying   Pde6brd1 allele
004202   B6.C3 Pde6brd1 Hps4le/+ +-Lmx1adr-8J/J
000002   B6.C3-Pde6brd1 Hps4le/J
001022   B6C3FeF1/J a/a
000652   BDP/J
000653   BUB/BnJ
002439   C3.129P2(B6)-B2mtm1Unc/J
005494   C3.129S1(B6)-Grm1rcw/J
000480   C3.MRL-Faslpr/J
001957   C3A Pde6brd1.O20/A-Prph2Rd2/J
005973   C3Bir.129P2(B6)-Il10C3Bir/LtJ
004326   C3Bir.129P2(B6)-Il10tm1Cgn/Lt
003968   C3Bir.129P2(B6)-Il10tm1Cgn/LtJ
001906   C3Ga.Cg-Catb/J
001904   C3H-Atcayji-hes/J
000659   C3H/HeJ
000784   C3H/HeJ-Faslgld/J
000509   C3H/HeJ-Lystbg-2J/J
002433   C3H/HeJ-Spnb4qv-lnd2J/J
005972   C3H/HeJBirLtJ
001824   C3H/HeJSxJ
000635   C3H/HeOuJ
000474   C3H/HeSn
001431   C3H/HeSn-ocd/J
000661   C3H/HeSnJ
002235   C3H/HeSnJ-Ctnna2cdf/J
002333   C3H/HeSnJ-gri/J
006435   C3HeB.SW-Soaa/MonJ
000658   C3HeB/FeJ
002588   C3HeB/FeJ-Eya1bor/J
001533   C3HeB/FeJ-Mc1rE-so Gli3Xt-J/J
001886   C3HeB/FeJLe a/a-gnd/J
001908   C3HfB/BiJ
001502   C3Sn.B6-Epha4rb/J
001547   C3Sn.Cg-Cm/J
000656   CBA/J
000813   CBA/J-Atp7aMo-pew/J
000660   DA/HuSnJ
000023   FL/1ReJ
000025   FL/4ReJ
003024   FVB.129P2(B6)-Fmr1tm1Cgr/J
002539   FVB.129P2-Abcb4tm1Bor/J
002935   FVB.129S2(B6)-Ccnd1tm1Wbg/J
002953   FVB.Cg-Tg(MMTVTGFA)254Rjc/J
003170   FVB.Cg-Tg(Myh6-tTA)6Smbf/J
003078   FVB.Cg-Tg(WapIgf1)39Dlr/J
003257   FVB/N-Tg(GFAPGFP)14Mes/J
002374   FVB/N-Tg(MMTV-PyVT)634Mul/J
002856   FVB/N-Tg(TIE2-lacZ)182Sato/J
002384   FVB/N-Tg(UcpDta)1Kz/J
001800   FVB/NJ
003487   FVB/NJ-Tg(XGFAP-lacZ)3Mes/J
001491   FVB/NMob
000734   MOLD/RkJ
000550   MOLF/EiJ
002423   NON/ShiLtJ
000679   P/J
000680   PL/J
100299   PLSJLF1/J
000269   SB/LeJ
005651   SJL.AK-Thy1a/TseJ
000686   SJL/J
000688   ST/bJ
004808   STOCK Mapttm1(EGFP)Klt Tg(MAPT)8cPdav/J
002648   STOCK a/a Cln6nclf/J
000279   STOCK gr +/+ Ap3d1mh/J
005965   STOCK Tg(Pomc1-cre)16Lowl/J
004770   SW.B6-Soab/J
002023   SWR.M-Emv21 Emv22/J
000689   SWR/J
000939   SWR/J-Clcn1adr-mto/J
000692   WB/ReJ KitW/J
100410   WBB6F1/J-KitW/KitW-v/J
000693   WC/ReJ KitlSl/J
100401   WCB6F1/J KitlSl KitlSl-d
View Strains carrying   Pde6brd1     (74 strains)

View Strains carrying other alleles of Pde6b     (8 strains)

Research Applications

This mouse can be used to support research in many areas including:

Atp7btx-J related

Metabolism Research

Mouse/Human Gene Homologs
Wilson disease

Pde6brd1 related
retinitis pigmentosa, autosomal recessive

Sensorineural Research
Retinal Degeneration

References

Additional References

Price and Supply Information

Strain Name: C3HeB/FeJ-Atp7btx-J/J
Stock Number: 001576

Price Details

IMPORTANT NOTE: Prices are based on shipping destination. The shipping destinations are:

*Pricing for Shipping Destination selected:

        USA, Canada and Mexico

Price(s) in US dollars ($)
Cryorecovery Fee $1900.00

Supply Details

Standard SupplyRepository-Cryopreserved. Must Be Recovered. Please refer to pricing and supply notes for further information.
Supply Notes Cryorecovery - Standard.
The recovery process begins when a signed agreement form is returned to the Customer Service Department after order placement. Although results vary by strain, at least two males and two females (two pairs) will be provided, typically within 15 weeks of our receipt of the signed agreement form. If the first recovery attempt is unsuccessful or only one pair is recovered, a second recovery will be done, extending the delivery time to approximately 25 weeks. At least one member of each pair will be of known genotype and will carry the mutation if it is a mutant strain. Please note that pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation. Price represents a repository maintenance fee, which includes the cost of recovery of the strain from the cryopreservation resource and the periodic replacement of the frozen embryos used for recovery.

Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
One to two pairs will be recovered to establish a Dedicated Supply of mice. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services: Tel: 1-800-422-6423 or 1-207-288-5845; Email: jaxservices@jax.org.
This strain is included in the Mouse Mutant Resource collection.
Genomic DNA is available for this strain from the Mouse DNA Resource.

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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.
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