Strain Name:

B6(Cg)-Cys1cpk/J

Stock Number:

000152

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Availability:

Cryopreserved - Ready for recovery

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

Former Names C57BL/6J-Cys1cpk/J    (Changed: 05-JUL-05 )
C57BL/6J-cpk    (Changed: 15-DEC-04 )
Type Mutant Strain; Spontaneous Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Specieslaboratory mouse
GenerationN36
Generation Definitions

Appearance
black
Related Genotype: a/a

Control Information

  Control
   Untyped from the colony
 
  Considerations for Choosing Controls

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Model with phenotypic similarity to human disease where etiologies are distinct. Human genes are associated with this disease. Orthologs of these genes do not appear in the mouse genotype(s).
Polycystic Kidney Disease, Autosomal Recessive; ARPKD
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Cys1cpk/Cys1+

        B6(Cg)-Cys1cpk/J
  • hearing/vestibular/ear phenotype
  • abnormal Reissner membrane morphology
    • the perilymphatic side of Reissner's membrane is transparent but the endolymphatic side is normal   (MGI Ref ID J:1001)
  • abnormal organ of Corti morphology
    • the organ of Corti is absent from the first cochlear turn but present in the second cochlear turn   (MGI Ref ID J:1001)
    • in the second cochlear turn the inner and outer hair cells are normal but debris is seen in Nuel's space and the outer pillar cells   (MGI Ref ID J:1001)
    • abnormal cochlear inner hair cell morphology
      • in the upper portion of the first turn of the cochlea, vesicle formation is seen in the inner hair cells   (MGI Ref ID J:1001)
    • abnormal pillar cell morphology
      • some inner pillar cells show a cystic formation at the base of the cell   (MGI Ref ID J:1001)
    • absent cochlear outer hair cells
      • in the upper portion of the first turn of the cochlea outer hair cells are absent   (MGI Ref ID J:1001)
  • increased or absent threshold for auditory brainstem response
    • hearing is impaired with auditory evoked potential indicating some hearing loss at 90 db   (MGI Ref ID J:1001)
  • nervous system phenotype
  • abnormal cochlear inner hair cell morphology
    • in the upper portion of the first turn of the cochlea, vesicle formation is seen in the inner hair cells   (MGI Ref ID J:1001)
  • absent cochlear outer hair cells
    • in the upper portion of the first turn of the cochlea outer hair cells are absent   (MGI Ref ID J:1001)
  • cochlear ganglion hypoplasia
    • few spiral ganglia are seen in the first cochlear turn   (MGI Ref ID J:1001)

Cys1cpk/Cys1cpk

        B6(Cg)-Cys1cpk/J
  • mortality/aging
  • complete postnatal lethality
    • die by 20-23 days of age   (MGI Ref ID J:6718)
    • die around 19 days of age   (MGI Ref ID J:31533)
  • renal/urinary system phenotype
  • abnormal kidney corticomedullary boundary morphology
    • large cysts distort the corticomedullary junction   (MGI Ref ID J:6718)
  • colorless urine
    • urine is colorless in grossly affected mice   (MGI Ref ID J:6718)
  • enlarged kidney
    • increase in kidney size is the result of both increased cystic fluid and increased cell mass   (MGI Ref ID J:9290)
    • at 23 days of age, kidneys occupy almost the entire abdominal cavity   (MGI Ref ID J:6718)
    • at 17-24 days of age, average kidneys size was 16 mm x 11 mm compared to 6 mm x 3 mm in wild-type mice   (MGI Ref ID J:6718)
    • increased kidney weight
      • kidney weight is increased in newborns and a progressive increase in kidney wet and dry weight as well as size is seen with age   (MGI Ref ID J:9290)
  • kidney cysts
    • initially presents as dilation of the proximal tubule in fetal and newborn mice, then shifts to dilation of the cortical and medullary collecting ducts after the first week of life   (MGI Ref ID J:9290)
    • epithelial hyperplasia but not polypoid hyperplasia is seen in the cysts   (MGI Ref ID J:9290)
    • large cysts occupy the entire kidney, distort the corticomedullary junction, involve collecting ducts and other portions of the kidney, and are lined with flattened epithelium the is frequently squamous   (MGI Ref ID J:6718)
    • polycystic kidney
      • bilateral polycystic kidneys become apparent by 8 - 9 days of age   (MGI Ref ID J:31533)
  • homeostasis/metabolism phenotype
  • colorless urine
    • urine is colorless in grossly affected mice   (MGI Ref ID J:6718)
  • increased blood urea nitrogen level
    • progressive azotemia seen starting at 1 week of age   (MGI Ref ID J:9290)
    • mean BUN was 120mg% compared to 0.2mg% in wild-type mice   (MGI Ref ID J:6718)
  • growth/size/body phenotype
  • distended abdomen
    • apparent by 8 - 9 days of age   (MGI Ref ID J:31533)
    • seen at 10-13 days of age   (MGI Ref ID J:6718)
  • hematopoietic system phenotype
  • decreased hematocrit
    • seen at 2 - 3 weeks of age   (MGI Ref ID J:9290)
  • behavior/neurological phenotype
  • lethargy
    • become progressively lethargic   (MGI Ref ID J:6718)

Cys1cpk/Cys1cpk

        B6(Cg)-Cys1cpk/JUnc
  • mortality/aging
  • premature death
    • most die by 4 weeks of age with a few rare survivors reaching 5 weeks of age   (MGI Ref ID J:7202)
  • renal/urinary system phenotype
  • colorless urine
    • at around 4 weeks of age urine becomes colorless   (MGI Ref ID J:7202)
  • enlarged kidney
    • at 3 weeks of age the kidney occupies almost the entire abdominal cavity and constitutes about 25% of body weight   (MGI Ref ID J:7202)
  • kidney cysts
    • cysts are grossly visible on cut surfaces of kidneys at 3 weeks of age with mild cystic changes already visible at E17   (MGI Ref ID J:7202)
    • progressive dilation of the proximal convoluted tubules and collecting ducts associated with intracellular vacuoles in proximal convoluted tubule cells   (MGI Ref ID J:7202)
  • homeostasis/metabolism phenotype
  • colorless urine
    • at around 4 weeks of age urine becomes colorless   (MGI Ref ID J:7202)
  • increased blood urea nitrogen level   (MGI Ref ID J:7202)
  • increased circulating creatinine level   (MGI Ref ID J:7202)
  • growth/size/body phenotype
  • distended abdomen
    • seen at 3 weeks of age   (MGI Ref ID J:7202)
  • behavior/neurological phenotype
  • lethargy
    • seen at around 4 weeks of age   (MGI Ref ID J:7202)
  • tremors
    • seen at around 4 weeks of age   (MGI Ref ID J:7202)

The following phenotype information is associated with a similar, but not exact match to this JAX® Mice strain.

Cys1cpk/Cys1+

        involves: C57BL/6J * CD-1
  • renal/urinary system phenotype
  • kidney cysts
    • proximal tubular cysts seen in 1 year old heterozygotes   (MGI Ref ID J:33739)
  • liver/biliary system phenotype
  • liver cysts
    • heptatic cysts lined with biliary epithelium are seen in 1 year old heterozygotes   (MGI Ref ID J:33739)
  • liver fibrosis
    • seen in 1 year old heterozygotes, associated with hepatic cysts   (MGI Ref ID J:33739)

Cys1cpk/Cys1+

        C.B6(Cg)-Cys1cpk
  • liver/biliary system phenotype
  • liver cysts
    • massive hepatic biliary ductal cysts are seen in 1 year old heterozygotes   (MGI Ref ID J:99616)
  • liver fibrosis
    • associated with liver cysts   (MGI Ref ID J:99616)

Cys1cpk/Cys1cpk

        involves: C57BL/6J * CD-1
  • mortality/aging
  • postnatal lethality
    • uremic mortality is seen in less than 10% of homozygotes at 14-20 days of age   (MGI Ref ID J:33739)
  • renal/urinary system phenotype
  • enlarged kidney
    • seen at 4 days of age   (MGI Ref ID J:33739)
  • kidney cysts
    • at 4 days of age proximal tubule cysts are seen with focal collecting duct dilations in the cortex and outer medulla and by 8-20 days of age collecting duct cysts expand to replace almost all the normal kidney tissue   (MGI Ref ID J:33739)
    • cells lining the proximal tubule cysts and later the collecting duct cysts appear immature   (MGI Ref ID J:33739)
  • homeostasis/metabolism phenotype
  • increased blood urea nitrogen level
    • azotemia seen at 12 days of age   (MGI Ref ID J:33739)
  • endocrine/exocrine gland phenotype
  • biliary cyst
    • all common bile ducts have cysts   (MGI Ref ID J:33739)
  • pancreas cysts
    • all pancreatic ducts have cysts   (MGI Ref ID J:33739)
  • thymus hypoplasia   (MGI Ref ID J:33739)
  • liver/biliary system phenotype
  • biliary cyst
    • all common bile ducts have cysts   (MGI Ref ID J:33739)
  • liver cysts
    • intrahepatic bile duct cysts consisting of focal dilations in the triad region through to the terminal intrahepatic branches without signs of significant periductal proliferation   (MGI Ref ID J:33739)
  • immune system phenotype
  • spleen hypoplasia   (MGI Ref ID J:33739)
  • thymus hypoplasia   (MGI Ref ID J:33739)
  • hematopoietic system phenotype
  • spleen hypoplasia   (MGI Ref ID J:33739)
  • thymus hypoplasia   (MGI Ref ID J:33739)

Cys1cpk/Cys1cpk

        C.B6(Cg)-Cys1cpk
  • renal/urinary system phenotype
  • enlarged kidney
    • rapid kidney enlargement after birth associated with development of cysts in the collecting ducts   (MGI Ref ID J:99616)
    • EGF treatment reduced kidney size but did not improve kidney function   (MGI Ref ID J:99616)
  • kidney cysts
    • after birth cysts shift to the collecting ducts and cysts are lined with a single layer of epithelial cells with polygonal principal-like cell appearance   (MGI Ref ID J:99616)
    • EGF treatment reduces the severity of kidney cysts   (MGI Ref ID J:99616)
    • kidney cortex cysts
      • newborns have cysts in the inner cortical proximal tubules   (MGI Ref ID J:99616)
  • homeostasis/metabolism phenotype
  • increased blood urea nitrogen level
    • significant by 10 days of age   (MGI Ref ID J:99616)
  • endocrine/exocrine gland phenotype
  • absent pancreatic islets
    • almost no islets of Langerhans seen   (MGI Ref ID J:99616)
  • biliary cyst
    • intrahepatic biliary duct cysts associated with peridcuctal hyperplasia and fibrosis   (MGI Ref ID J:99616)
  • dilated bile duct
    • common bile duct dilation   (MGI Ref ID J:99616)
    • EGF treatment reduces bile duct dilation   (MGI Ref ID J:99616)
  • dilated pancreatic duct
    • dilation of the pancreatic ducts present in newborns   (MGI Ref ID J:99616)
  • pancreas fibrosis
    • periductal fibrosis   (MGI Ref ID J:99616)
  • liver/biliary system phenotype
  • biliary cyst
    • intrahepatic biliary duct cysts associated with peridcuctal hyperplasia and fibrosis   (MGI Ref ID J:99616)
  • dilated bile duct
    • common bile duct dilation   (MGI Ref ID J:99616)
    • EGF treatment reduces bile duct dilation   (MGI Ref ID J:99616)
  • liver fibrosis
    • peridcuctal fibrosis associated with intrahepatic biliary duct cysts   (MGI Ref ID J:99616)
  • growth/size/body phenotype
  • decreased body size
    • seen at 5 and 15 days of age   (MGI Ref ID J:99616)
  • digestive/alimentary phenotype
  • dilated pancreatic duct
    • dilation of the pancreatic ducts present in newborns   (MGI Ref ID J:99616)

Cys1cpk/Cys1cpk

        D2J.B6(Cg)-Cys1cpk
  • renal/urinary system phenotype
  • enlarged kidney
    • kidneys are grossly enlarged   (MGI Ref ID J:99620)
    • increased kidney weight
      • kidney wet weight is increased to 1.96g - 2.12g compared to 0.16g - 0.19g in wild-type mice   (MGI Ref ID J:99620)
  • kidney cysts
    • multiple cysts of varying size that obscure the demarcation between the cortex and medulla   (MGI Ref ID J:99620)
    • cysts become larger with age and are initially found in the proximal tubules epithelial intercellular spaces are often focally ballooned   (MGI Ref ID J:99620)
  • homeostasis/metabolism phenotype
  • increased blood urea nitrogen level
    • mean BUN is 143mg% compared to 21mg% in wild-type mice   (MGI Ref ID J:99620)
  • increased circulating creatinine level
    • mean creatinine is 0.66mg% compared to 0.23mg% in wild-type mice   (MGI Ref ID J:99620)
  • endocrine/exocrine gland phenotype
  • abnormal bile duct morphology
    • bile ductules are somewhat enlarged   (MGI Ref ID J:99620)
  • abnormal pancreatic acinus morphology
    • poorly-formed acini are connected to enlarged, dilated ducts   (MGI Ref ID J:99620)
    • ectopic pancreatic acinar cells
      • exocrine cells containing zygomen granules are seen in clusters unassociated with well-formed acini   (MGI Ref ID J:99620)
  • absent pancreatic islets
    • few if any well-formed islets are found   (MGI Ref ID J:99620)
  • dilated pancreatic duct
    • poorly-formed acini are connected to enlarged, dilated ducts   (MGI Ref ID J:99620)
  • pancreas fibrosis
    • normal architecture is replaced by sheets of fibrous tissue   (MGI Ref ID J:99620)
  • liver/biliary system phenotype
  • abnormal bile duct morphology
    • bile ductules are somewhat enlarged   (MGI Ref ID J:99620)
  • liver fibrosis
    • some portal areas have increased fibrous tissue   (MGI Ref ID J:99620)
  • behavior/neurological phenotype
  • abnormal suckling behavior
    • feed poorly during the first few hours of life and generally fail to thrive   (MGI Ref ID J:99620)
  • lethargy   (MGI Ref ID J:99620)
  • digestive/alimentary phenotype
  • abnormal pancreatic acinus morphology
    • poorly-formed acini are connected to enlarged, dilated ducts   (MGI Ref ID J:99620)
    • ectopic pancreatic acinar cells
      • exocrine cells containing zygomen granules are seen in clusters unassociated with well-formed acini   (MGI Ref ID J:99620)
  • dilated pancreatic duct
    • poorly-formed acini are connected to enlarged, dilated ducts   (MGI Ref ID J:99620)

Cys1cpk/Cys1cpk

        129.B6-Cys1cpk
  • renal/urinary system phenotype
  • increased kidney weight   (MGI Ref ID J:116210)
  • kidney cysts   (MGI Ref ID J:116210)
  • liver/biliary system phenotype
  • liver inflammation   (MGI Ref ID J:116210)
  • endocrine/exocrine gland phenotype
  • pancreas cysts   (MGI Ref ID J:116210)
  • immune system phenotype
  • liver inflammation   (MGI Ref ID J:116210)
View Research Applications

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

Cys1cpk related

Developmental Biology Research
Internal/Organ Defects
      kidney

Internal/Organ Research
Kidney Defects
      polycystic kidney disease

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Cys1cpk
Allele Name congenital polycystic kidneys
Allele Type Spontaneous
Common Name(s) ck; cpk;
Strain of OriginB6.Cg-Bloc1s6
Gene Symbol and Name Cys1, cystin 1
Chromosome 12
Gene Common Name(s) 2900006B19Rik; AV218859; RIKEN cDNA 2900006B19 gene; ck; congenital polycystic kidneys; cpk; cystic kidneys; expressed sequence AV218859;
Molecular Note The underlying mutation responsible for the phenotype in the cpk mouse consists of a tandem deletion of 12 bp and 19 bp. The result is a frame-shift within exon 1 that truncates the protein. Northern analysis from kidney and liver revealed the presenceof transcript in homozygous mutant animals at reduced levels compared to wild-type. [MGI Ref ID J:74701]

Genotyping

Genotyping Information

Genotyping Protocols

Cys1cpk, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Additional References

Woo D; Lee GY; Anderson E; Aziz N. 2001. Immature ovaries and polycystic kidneys in the congenital polycystic kidney mouse may be due to abnormal sex steroid metabolism. Mol Cell Endocrinol 176(1-2):155-62. [PubMed: 11369455]  [MGI Ref ID J:82596]

Cys1cpk related

Alcalay NI; Sharma M; Vassmer D; Chapman B; Paul B; Zhou J; Brantley JG; Wallace DP; Maser RL; Vanden Heuvel GB. 2008. Acceleration of polycystic kidney disease progression in cpk mice carrying a deletion in the homeodomain protein Cux1. Am J Physiol Renal Physiol 295(6):F1725-34. [PubMed: 18829740]  [MGI Ref ID J:142805]

Ali SM; Wong VY; Kikly K; Fredrickson TA; Keller PM; DeWolf WE Jr; Lee D; Brooks DP. 2000. Apoptosis in polycystic kidney disease: involvement of caspases. Am J Physiol Regul Integr Comp Physiol 278(3):R763-9. [PubMed: 10712299]  [MGI Ref ID J:114279]

Atencio IA; Villarreal LP. 1994. Polyomavirus replicates in differentiating but not in proliferating tubules of adult mouse polycystic kidneys. Virology 201(1):26-35. [PubMed: 8178487]  [MGI Ref ID J:17871]

Avner ED. 1993. Epithelial polarity and differentiation in polycystic kidney disease. J Cell Sci Suppl 17:217-22. [PubMed: 8144700]  [MGI Ref ID J:18532]

Aziz N; Anderson E; Lee GY; Woo DD. 2001. Arrested testis development in the cpk mouse may be the result of abnormal steroid metabolism. Mol Cell Endocrinol 171(1-2):83-8. [PubMed: 11165015]  [MGI Ref ID J:67183]

Aziz N; Brown D; Lee WS; Naray-Fejes-Toth A. 1996. Aberrant 11beta-hydroxysteroid dehydrogenase-1 activity in the cpk mouse: implications for regulation by the Ke 6 gene. Endocrinology 137(12):5581-8. [PubMed: 8940387]  [MGI Ref ID J:37226]

Aziz N; Maxwell MM; Brenner BM. 1994. Coordinate regulation of 11 beta-HSD and Ke 6 genes in cpk mouse: implications for steroid metabolic defect in PKD. Am J Physiol 267(5 Pt 2):F791-7. [PubMed: 7977782]  [MGI Ref ID J:21186]

Belibi F; Zafar I; Ravichandran K; Segvic AB; Jani A; Ljubanovic DG; Edelstein CL. 2011. Hypoxia-inducible factor-1{alpha} (HIF-1{alpha}) and autophagy in polycystic kidney disease (PKD). Am J Physiol Renal Physiol 300(5):F1235-43. [PubMed: 21270095]  [MGI Ref ID J:171475]

Chiu MG; Johnson TM; Woolf AS; Dahm-Vicker EM; Long DA; Guay-Woodford L; Hillman KA; Bawumia S; Venner K; Hughes RC; Poirier F; Winyard PJ. 2006. Galectin-3 associates with the primary cilium and modulates cyst growth in congenital polycystic kidney disease. Am J Pathol 169(6):1925-38. [PubMed: 17148658]  [MGI Ref ID J:116210]

Cho H; Buchanan J; Strong D; Yamada Y; Yoo TJ. 1991. The molecular and structural basis of hearing impairment in mice with the cpk mutant gene. Ann N Y Acad Sci 630:262-4. [PubMed: 1952600]  [MGI Ref ID J:1001]

Davisson MT; Guay-Woodford LM; Harris HW; D'Eustachio P. 1991. The mouse polycystic kidney disease mutation (cpk) is located on proximal chromosome 12. Genomics 9(4):778-81. [PubMed: 2037305]  [MGI Ref ID J:11200]

Doctor RB; Johnson S; Brodsky KS; Amura CR; Gattone V; Fitz JG. 2007. Regulated ion transport in mouse liver cyst epithelial cells. Biochim Biophys Acta 1772(3):345-54. [PubMed: 17208416]  [MGI Ref ID J:121384]

Fry JL Jr; Koch WE; Jennette JC; McFarland E; Fried FA; Mandell J. 1985. A genetically determined murine model of infantile polycystic kidney disease. J Urol 134(4):828-33. [PubMed: 4032601]  [MGI Ref ID J:99620]

Galarreta CI; Grantham JJ; Forbes MS; Maser RL; Wallace DP; Chevalier RL. 2014. Tubular obstruction leads to progressive proximal tubular injury and atubular glomeruli in polycystic kidney disease. Am J Pathol 184(7):1957-66. [PubMed: 24815352]  [MGI Ref ID J:211661]

Gattone VH 2nd; Andrews GK; Niu FW; Chadwick LJ; Klein RM; Calvet JP. 1990. Defective epidermal growth factor gene expression in mice with polycystic kidney disease. Dev Biol 138(1):225-30. [PubMed: 1968405]  [MGI Ref ID J:10330]

Gattone VH 2nd; Calvet JP; Cowley BD Jr; Evan AP; Shaver TS; Helmstadter K; Grantham JJ. 1988. Autosomal recessive polycystic kidney disease in a murine model. A gross and microscopic description. Lab Invest 59(2):231-8. [PubMed: 3404974]  [MGI Ref ID J:9290]

Gattone VH 2nd; Lowden DA; Cowley BD Jr. 1995. Epidermal growth factor ameliorates autosomal recessive polycystic kidney disease in mice. Dev Biol 169(2):504-10. [PubMed: 7781894]  [MGI Ref ID J:26131]

Gattone VH 2nd; MacNaughton KA; Kraybill AL. 1996. Murine autosomal recessive polycystic kidney disease with multiorgan involvement induced by the cpk gene. Anat Rec 245(3):488-99. [PubMed: 8800407]  [MGI Ref ID J:33739]

Gattone VH 2nd; Maser RL; Tian C; Rosenberg JM; Branden MG. 1999. Developmental expression of urine concentration-associated genes and their altered expression in murine infantile-type polycystic kidney disease. Dev Genet 24(3-4):309-18. [PubMed: 10322639]  [MGI Ref ID J:54490]

Gattone VH; Ricker JL; Trambaugh CM; Klein RM. 2002. Multiorgan mRNA misexpression in murine autosomal recessive polycystic kidney disease. Kidney Int 62(5):1560-9. [PubMed: 12371956]  [MGI Ref ID J:104014]

Guay-Woodford LM. 2003. Murine models of polycystic kidney disease: molecular and therapeutic insights. Am J Physiol Renal Physiol 285(6):F1034-49. [PubMed: 14600027]  [MGI Ref ID J:87130]

Guay-Woodford LM; Green WJ; Lindsey JR; Beier DR. 2000. Germline and somatic loss of function of the mouse cpk gene causes biliary ductal pathology that is genetically modulated. Hum Mol Genet 9(5):769-78. [PubMed: 10749984]  [MGI Ref ID J:61399]

Harding MA; Chadwick LJ; Gattone VH 2nd; Calvet JP. 1991. The SGP-2 gene is developmentally regulated in the mouse kidney and abnormally expressed in collecting duct cysts in polycystic kidney disease. Dev Biol 146(2):483-90. [PubMed: 1864465]  [MGI Ref ID J:11365]

Harding MA; Gattone VH 2nd; Grantham JJ; Calvet JP. 1992. Localization of overexpressed c-myc mRNA in polycystic kidneys of the cpk mouse. Kidney Int 41(2):317-25. [PubMed: 1552705]  [MGI Ref ID J:543]

Hou X; Mrug M; Yoder BK; Lefkowitz EJ; Kremmidiotis G; D'Eustachio P; Beier DR; Guay-Woodford LM. 2002. Cystin, a novel cilia-associated protein, is disrupted in the cpk mouse model of polycystic kidney disease. J Clin Invest 109(4):533-40. [PubMed: 11854326]  [MGI Ref ID J:74701]

Hulea L; Nepveu A. 2012. CUX1 transcription factors: from biochemical activities and cell-based assays to mouse models and human diseases. Gene 497(1):18-26. [PubMed: 22306263]  [MGI Ref ID J:183683]

Lakshmanan J; Fisher DA. 1993. An inborn error in epidermal growth factor prohormone metabolism in a mouse model of autosomal recessive polycystic kidney disease. Biochem Biophys Res Commun 196(2):892-901. [PubMed: 8240367]  [MGI Ref ID J:15417]

Mandell J; Koch WK; Nidess R; Preminger GM; McFarland E. 1983. Congenital polycystic kidney disease. Genetically transmitted infantile polycystic kidney disease in C57BL/6J mice. Am J Pathol 113(1):112-4. [PubMed: 6624875]  [MGI Ref ID J:7202]

Mrug M; Zhou J; Woo Y; Cui X; Szalai AJ; Novak J; Churchill GA; Guay-Woodford LM. 2008. Overexpression of innate immune response genes in a model of recessive polycystic kidney disease. Kidney Int 73(1):63-76. [PubMed: 17960140]  [MGI Ref ID J:135367]

Muchatuta MN; Gattone VH 2nd; Witzmann FA; Blazer-Yost BL. 2009. Structural and functional analyses of liver cysts from the BALB/c-cpk mouse model of polycystic kidney disease. Exp Biol Med (Maywood) 234(1):17-27. [PubMed: 18997107]  [MGI Ref ID J:165008]

Nakamura T; Ebihara I; Fukui M; Osada S; Tomino Y; Masaki T; Goto K; Furuichi Y; Koide H. 1993. Increased endothelin and endothelin receptor mRNA expression in polycystic kidneys of cpk mice. J Am Soc Nephrol 4(4):1064-72. [PubMed: 7904485]  [MGI Ref ID J:18219]

Ogborn MR; Sareen S; Tomobe K; Takahashi H; Crocker JF. 1995. Renal tubule Na,K-ATPase polarity in different animal models of polycystic kidney disease. J Histochem Cytochem 43(8):785-90. [PubMed: 7622841]  [MGI Ref ID J:27468]

Ojeda JL. 1999. Abnormal tenascin expression in murine autosomal recessive polycystic kidneys. Nephron 82(3):261-9. [PubMed: 10395999]  [MGI Ref ID J:57379]

Orellana SA; Sweeney WE; Neff CD; Avner ED. 1995. Epidermal growth factor receptor expression is abnormal in murine polycystic kidney. Kidney Int 47(2):490-9. [PubMed: 7723235]  [MGI Ref ID J:23046]

Ostrom L; Tang MJ; Gruss P; Dressler GR. 2000. Reduced Pax2 gene dosage increases apoptosis and slows the progression of renal cystic disease. Dev Biol 219(2):250-8. [PubMed: 10694420]  [MGI Ref ID J:61157]

Preminger GM; Koch WE; Fried FA; McFarland E; Murphy ED; Mandell J. 1982. Murine congenital polycystic kidney disease: a model for studying development of cystic disease. J Urol 127(3):556-60. [PubMed: 7062441]  [MGI Ref ID J:6718]

Ribacchi R. 1975. [Congenital polycystic kidney in PM/Se mice] Lav Ist Anat Istol Patol Univ Studi Perugia 35(3):81-8. [PubMed: 1232513]  [MGI Ref ID J:15028]

Ricker JL; Gattone VH 2nd; Calvet JP; Rankin CA. 2000. Development of autosomal recessive polycystic kidney disease in BALB/c-cpk/cpk mice. J Am Soc Nephrol 11(10):1837-47. [PubMed: 11004214]  [MGI Ref ID J:99616]

Rocco MV; Neilson EG; Hoyer JR; Ziyadeh FN. 1992. Attenuated expression of epithelial cell adhesion molecules in murine polycystic kidney disease. Am J Physiol 262(4 Pt 2):F679-86. [PubMed: 1566881]  [MGI Ref ID J:574]

Russell ES; McFarland EC. 1977. Cystic kidneys, ck Mouse News Lett 56:40.  [MGI Ref ID J:31533]

Takahashi H; Calvet JP; Dittemore-Hoover D; Yoshida K; Grantham JJ; Gattone VH 2nd. 1991. A hereditary model of slowly progressive polycystic kidney disease in the mouse. J Am Soc Nephrol 1(7):980-9. [PubMed: 1883968]  [MGI Ref ID J:11399]

Tao Y; Zafar I; Kim J; Schrier RW; Edelstein CL. 2008. Caspase-3 gene deletion prolongs survival in polycystic kidney disease. J Am Soc Nephrol 19(4):749-55. [PubMed: 18272845]  [MGI Ref ID J:149924]

Woo D; Lee GY; Anderson E; Aziz N. 2001. Immature ovaries and polycystic kidneys in the congenital polycystic kidney mouse may be due to abnormal sex steroid metabolism. Mol Cell Endocrinol 176(1-2):155-62. [PubMed: 11369455]  [MGI Ref ID J:82596]

Zhou J; Ouyang X; Cui X; Schoeb TR; Smythies LE; Johnson MR; Guay-Woodford LM; Chapman AB; Mrug M. 2010. Renal CD14 expression correlates with the progression of cystic kidney disease. Kidney Int 78(6):550-60. [PubMed: 20555320]  [MGI Ref ID J:184258]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200.

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


Pricing for USA, Canada and Mexico shipping destinations View International Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $3300.00
Animals Provided

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.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery of Strains Needing Progeny Testing
    At least two untested males and two untested females (two pairs) will be recovered (eight or more mice is typical). The total number of animals provided, their gender and genotype will vary. Untested animals typically are available to ship between 10 and 14 weeks from the date of your order. If the first recovery attempt is unsuccessful, a second recovery will be done, extending the overall recovery time to approximately 25 weeks. Progeny testing is required to identify the genotype of mice of this strain, as a genotyping assay is not available. This type of testing involves breeding the recovered animals and assessing the phenotype of the offspring in order to identify animals carrying the mutation of interest. We can perform the progeny testing for you as a service or we can ship all recovered animals to you for progeny testing at your facility. If you perform the progeny testing, there is no guarantee that a carrier will be identified. If we perform progeny testing as a service, additional breeding time will be required. In this case, when a male and female (one pair) are identified that carry the mutation, they and their offspring will be shipped. Delivery time for strains requiring progeny testing often exceeds 25 weeks and may take 12 months or more due to the difficulties in breeding some strains. The progeny testing cost is in addition to the recovery cost and is based on the number of boxes used and the time taken to produce the mice identified as carrying the mutation.
    Please note that identified 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.

    Please contact Customer Service for more information on the cost of progeny testing for a strain, tel: 1-800-422-6423 or 1-207-288-5845 (from any location). The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
    Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $4290.00
Animals Provided

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.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery of Strains Needing Progeny Testing
    At least two untested males and two untested females (two pairs) will be recovered (eight or more mice is typical). The total number of animals provided, their gender and genotype will vary. Untested animals typically are available to ship between 10 and 14 weeks from the date of your order. If the first recovery attempt is unsuccessful, a second recovery will be done, extending the overall recovery time to approximately 25 weeks. Progeny testing is required to identify the genotype of mice of this strain, as a genotyping assay is not available. This type of testing involves breeding the recovered animals and assessing the phenotype of the offspring in order to identify animals carrying the mutation of interest. We can perform the progeny testing for you as a service or we can ship all recovered animals to you for progeny testing at your facility. If you perform the progeny testing, there is no guarantee that a carrier will be identified. If we perform progeny testing as a service, additional breeding time will be required. In this case, when a male and female (one pair) are identified that carry the mutation, they and their offspring will be shipped. Delivery time for strains requiring progeny testing often exceeds 25 weeks and may take 12 months or more due to the difficulties in breeding some strains. The progeny testing cost is in addition to the recovery cost and is based on the number of boxes used and the time taken to produce the mice identified as carrying the mutation.
    Please note that identified 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.

    Please contact Customer Service for more information on the cost of progeny testing for a strain, tel: 1-800-422-6423 or 1-207-288-5845 (from any location). The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
    Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

General Supply Notes

  • View the complete collection of spontaneous mutants in the Mouse Mutant Resource.

Control Information

  Control
   Untyped from the colony
 
  Considerations for Choosing Controls
  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 Information
JAX® Mice
Surgical and Preconditioning Services
JAX® Services
Customer Services and Support
Tel: 1-800-422-6423 or 1-207-288-5845
Fax: 1-207-288-6150
Technical Support Email Form

Terms of Use

Terms of Use


General Terms and Conditions


Contact information

General inquiries regarding Terms of Use

Contracts Administration

phone:207-288-6470

JAX® Mice, Products & Services Conditions of Use

"MICE" means mouse strains, their progeny derived by inbreeding or crossbreeding, unmodified derivatives from mouse strains or their progeny supplied by The Jackson Laboratory ("JACKSON"). "PRODUCTS" means biological materials supplied by JACKSON, and their derivatives. "RECIPIENT" means each recipient of MICE, PRODUCTS, or services provided by JACKSON including each institution, its employees and other researchers under its control. MICE or PRODUCTS shall not be: (i) used for any purpose other than the internal research, (ii) sold or otherwise provided to any third party for any use, or (iii) provided to any agent or other third party to provide breeding or other services. Acceptance of MICE or PRODUCTS from JACKSON shall be deemed as agreement by RECIPIENT to these conditions, and departure from these conditions requires JACKSON's prior written authorization.

No Warranty

MICE, PRODUCTS AND SERVICES ARE PROVIDED “AS IS”. JACKSON EXTENDS NO WARRANTIES OF ANY KIND, EITHER EXPRESS, IMPLIED, OR STATUTORY, WITH RESPECT TO MICE, PRODUCTS OR SERVICES, INCLUDING ANY IMPLIED WARRANTY OF MERCHANTABILITY OR FITNESS FOR A PARTICULAR PURPOSE, OR ANY WARRANTY OF NON-INFRINGEMENT OF ANY PATENT, TRADEMARK, OR OTHER INTELLECTUAL PROPERTY RIGHTS.

In case of dissatisfaction for a valid reason and claimed in writing by a purchaser within ninety (90) days of receipt of mice, products or services, JACKSON will, at its option, provide credit or replacement for the mice or product received or the services provided.

No Liability

In no event shall JACKSON, its trustees, directors, officers, employees, and affiliates be liable for any causes of action or damages, including any direct, indirect, special, or consequential damages, arising out of the provision of MICE, PRODUCTS or services, including economic damage or injury to property and lost profits, and including any damage arising from acts or negligence on the part of JACKSON, its agents or employees. Unless prohibited by law, in purchasing or receiving MICE, PRODUCTS or services from JACKSON, purchaser or recipient, or any party claiming by or through them, expressly releases and discharges JACKSON from all such causes of action or damages, and further agrees to defend and indemnify JACKSON from any costs or damages arising out of any third party claims.

MICE and PRODUCTS are to be used in a safe manner and in accordance with all applicable governmental rules and regulations.

The foregoing represents the General Terms and Conditions applicable to JACKSON’s MICE, PRODUCTS or services. In addition, special terms and conditions of sale of certain MICE, PRODUCTS or services may be set forth separately in JACKSON web pages, catalogs, price lists, contracts, and/or other documents, and these special terms and conditions shall also govern the sale of these MICE, PRODUCTS and services by JACKSON, and by its licensees and distributors.

Acceptance of delivery of MICE, PRODUCTS or services shall be deemed agreement to these terms and conditions. No purchase order or other document transmitted by purchaser or recipient that may modify the terms and conditions hereof, shall be in any way binding on JACKSON, and instead the terms and conditions set forth herein, including any special terms and conditions set forth separately, shall govern the sale of MICE, PRODUCTS or services by JACKSON.


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