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Strain Name:

FVB.129P2-Abcb4tm1Bor/J

Stock Number:

002539

Availability:

Repository-Cryopreserved

Price and Supply Information

General Terms and Conditions

Genes & Alleles   Abcb4;   Abcb4tm1Bor;   Pde6b;   Pde6brd1;

Product Information

Strain Details

Type JAX® GEMM® Strain - Congenic
Additional information on JAX® GEMM® Strains.
Type JAX® GEMM® Strain - Mutant Strain
Type JAX® GEMM® Strain - Targeted Mutation
Specieslaboratory mouse
Background Strain FVB/N
Donor Strain 129P2 via E14TG2a ES cell line
Donating Investigator Piet Borst,   The Netherlands Cancer Institute
GenerationN6F10pN1 (09-NOV-05)

Appearance
albino
Related Genotype: Tyrc/Tyrc

Important Note
This strain is homozygous for the retinal degeneration allele Pde6brd1.

Strain Description
Mice homozygous for the Abcb4tm1Bor mutation lack the ability to secrete phospholipid into the bile from the liver. They develop a degenerative liver disease. Portal inflammation ensues at an early age followed by hepatocellular carcinoma development after the age of 1 year. Bile from heterozygous mice has half the level of phospholipid when compared to bile from homozygotes. No liver pathology has been shown in heterozygotes.

Mammalian Phenotype Terms assigned by genotype

The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.

Abcb4tm1Bor/Abcb4tm1Bor

        either: (involves: 129P2/OlaHsd) or (involves: 129P2/OlaHsd * FVB/N)
  • homeostasis/metabolism phenotype
  • abnormal blood chemistry (MGI Ref ID J:15531)
    • serum is yellowish, suggesting liver damage
    • abnormal circulating alanine transaminase level (MGI Ref ID J:15531)
      • 5.8-fold increase in alanine transaminase level
    • abnormal circulating alkaline phosphatase level (MGI Ref ID J:15531)
      • 3-fold increase in serum alkaline phosphatase level
    • abnormal circulating aspartate transaminase level (MGI Ref ID J:15531)
      • 4-fold increase in aspartate transaminase level
    • abnormal circulating bilirubin level (MGI Ref ID J:15531)
      • 12-fold elevated bilirubin level; ~50% of this is conjugated
  • immune system phenotype
  • bile duct inflammation (MGI Ref ID J:15531)
    • inflammation of bile ducts
  • liver inflammation (MGI Ref ID J:15531)
    • portal inflammation with mixed inflammatory infiltrate and slight fibrosis
  • liver/biliary system phenotype
  • abnormal bile composition (MGI Ref ID J:15531)
    • absence of phospholipid secretion in bile, lipid secretion is decreased and there is a 15-reduction in cholesterol secretion in bile
    • glutathione secretion in bile is ~14% of wild type
    • chloride secretion is slightly, but significantly, elevated
    • phospolipid to bile salt ratio is only ~41% of wild type
  • abnormal bile duct morphology (MGI Ref ID J:15531)
    • prominent widening and increased tortuosity of bile canaliculi with loss of microvilli is present
    • portal expansion due to ductular proliferation is seen
  • abnormal bile secretion (MGI Ref ID J:15531)
  • abnormal hepatocyte morphology (MGI Ref ID J:15531)
    • hepatocyte degeneration, irregular size with nuclear polymorphism and focal necrosis are observed, along with increased proliferation
  • bile duct dilation (MGI Ref ID J:15531)
    • larger bile ducts are dilated
  • bile duct inflammation (MGI Ref ID J:15531)
    • inflammation of bile ducts
  • intrahepatic cholestasis (MGI Ref ID J:15531)
    • modesrate liver damage with cholestatic properties (raised serum-conjugated bilirubin) is observed
  • liver inflammation (MGI Ref ID J:15531)
    • portal inflammation with mixed inflammatory infiltrate and slight fibrosis

Abcb4tm1Bor/Abcb4tm1Bor

        involves: 129P2/OlaHsd * FVB
  • homeostasis/metabolism phenotype
  • abnormal circulating enzyme level (MGI Ref ID J:114411)
    • increased circulating alanine transaminase level (MGI Ref ID J:114411)
    • increased circulating aspartate transaminase level (MGI Ref ID J:114411)
  • abnormal lipid homeostasis (MGI Ref ID J:114411)
    • decreased cholesterol absorption (MGI Ref ID J:114411)
      • 40% reduction in absorption efficiency
    • decreased circulating cholesterol level (MGI Ref ID J:114411)
      • plasma levels reduced
  • increased circulating bilirubin level (MGI Ref ID J:114411)
  • digestive/alimentary phenotype
  • decreased cholesterol absorption (MGI Ref ID J:114411)
    • 40% reduction in absorption efficiency

Gene & Allele Details

Allele Symbol Abcb4tm1Bor
Allele Name targeted mutation 1, Piet Borst
Common Name(s) Pgy2tm1Bor; mdr2-; mdr2neo1;
Mutation Made By Piet Borst,   The Netherlands Cancer Institute
Strain of Origin129P2/OlaHsd
ES Cell Line NameE14
ES Cell Line Strain129P2/OlaHsd
Gene Symbol and Name Abcb4, ATP-binding cassette, sub-family B (MDR/TAP), member 4
Chromosome 5
Gene Common Name(s) ABC21; GBD1; MDR2; MDR2/3; MDR3; P glycoprotein 2; PFIC-3; PGY3; Pgy-2; Pgy2; mdr-2;
Molecular Note A neomycin selection cassette replaced a genomic fragment containing exons 1 and 2. The translation initiation site is in exon 2. RNase proteiction assays indicated that mutant truncated transcripts are produced from this allele that contain exons 3-6 or 4-6. [MGI Ref ID J:15531]
 
Allele Symbol Pde6brd1
Allele Name retinal degeneration 1
Common Name(s) rd; rd-1; rd1; rodless retina;

Control Information

  Allele   Control
 Abcb4tm1Bor  001800 FVB/NJ
 
  Considerations for Choosing Controls
  Control Pricing Information for JAX® GEMM® Strains

Genotyping Protocols

Abcb4tm1Bor

Colony Maintenance

Breeding & HusbandryThe strain is maintained by mating homozygous siblings. Some health problems due to liver degeneration begin to arise at about 8 months of age. Expected coat color from breeding:Albino
Diet Information LabDiet® 5K52/5K67

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
001576   C3HeB/FeJ-Atp7btx-J/J
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
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)

Additional Web Information

Congenic Nomenclature

Research Applications

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

Cancer Research
Increased Tumor Incidence (Hepatomas: hepatacellular carcinoma)

Abcb4tm1Bor related

Cancer Research
Toxicology

Internal/Organ Research
Liver Defects

Metabolism Research

Research Tools
Toxicology Research

Pde6brd1 related

Mouse/Human Gene Homologs
retinitis pigmentosa, autosomal recessive

Sensorineural Research
Retinal Degeneration

References

Selected Reference(s)

Smit JJ; Schinkel AH; Oude Elferink RP; Groen AK; Wagenaar E; van Deemter L; Mol CA; Ottenhoff R; van der Lugt NM; van Roon MA; van der Valk MA; Offerhaus GJ; Berns AJ; Borst P. 1993. Homozygous disruption of the murine mdr2 P-glycoprotein gene leads to a complete absence of phospholipid from bile and to liver disease. Cell 75(3):451-62. [PubMed: 8106172]  [MGI Ref ID J:15531]

Additional References

Price and Supply Information

Strain Name: FVB.129P2-Abcb4tm1Bor/J
Stock Number: 002539

Price Details

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

*Pricing for Shipping Destination selected:

        International

Price(s) in US dollars ($)
Cryorecovery Fee $2470.00

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 Induced Mutant Resource Colony 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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