Strain Name:

129-Col4a3tm1Dec/J

Stock Number:

002908

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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 Mutant Strain; Targeted Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Specieslaboratory mouse
 
Donating InvestigatorDr. Dominic Cosgrove,   Boys Town National Research Hospital

Appearance
white-bellied agouti
Related Genotype: Aw/Aw

pink-eyed, light-bellied, light chinchilla
Related Genotype: Aw/Aw Oca2p Tyrc-ch/Oca2p Tyrc

albino
Related Genotype: Aw/Aw Oca2p Tyrc/Oca2p Tyrc

Description
Mice homozygous for the Col4a3tm1Dec targeted mutation develop glomerulonephritis and die at about 8.5 weeks of age. Survival time of homozygous mutant mice is extended to about 14 weeks of age in mice maintained on a mixed genetic background.

Control Information

  Control
   Wild-type from the colony
   000691 129X1/SvJ
 
  Considerations for Choosing Controls

Additional Web Information

New 129 Nomenclature Bulletin

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Model with phenotypic similarity to human disease where etiologies involve orthologs. Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).
Alport Syndrome, Autosomal Recessive
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Alport Syndrome, Autosomal Dominant   (COL4A3)
Hematuria, Benign Familial; BFH   (COL4A3)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Col4a3tm1Dec/Col4a3tm1Dec

        129X1/SvJ-Col4a3tm1Dec
  • hearing/vestibular/ear phenotype
  • abnormal cochlear basement membrane morphology
    • as early as 6 weeks, homozygotes display significant thickening of the strial capillary basement membrane (SCBM)   (MGI Ref ID J:91619)
    • at 7-9 weeks of age (i.e. prior to end-stage renal disease), SCBM thickening is associated with significantly elevated matrix metalloproteinases (MMP) deposition   (MGI Ref ID J:98449)
    • treatment with an inhibitor of MMP-2, -9, -12, and -14 exacerbates SCBM thickening, directly implicating altered basement membrane metabolism in maintaining normal SCBM composition and thickness   (MGI Ref ID J:98449)
  • abnormal stria vascularis morphology
    • at 7-9 weeks of age (i.e. prior to end-stage renal disease), homozygotes show matrix metalloproteinase dysregulation in the stria vascularis   (MGI Ref ID J:98449)
    • abnormal stria vascularis vasculature morphology
      • as early as 6 weeks, homozygotes display significant thickening of the strial capillary basement membrane (SCBM)   (MGI Ref ID J:91619)
  • increased or absent threshold for auditory brainstem response
    • at 6-8 weeks of age, auditory-evoked brainstem response measurements suggest a small increase in auditory thresholds across all frequencies tested with successive measurements on individual mutant mice   (MGI Ref ID J:91619)
  • sensorineural hearing loss
    • homozygotes display a moderate, high frequency, progressive sensorineural hearing loss   (MGI Ref ID J:91619)
  • cardiovascular system phenotype
  • abnormal stria vascularis vasculature morphology
    • as early as 6 weeks, homozygotes display significant thickening of the strial capillary basement membrane (SCBM)   (MGI Ref ID J:91619)
  • cellular phenotype
  • abnormal cochlear basement membrane morphology
    • as early as 6 weeks, homozygotes display significant thickening of the strial capillary basement membrane (SCBM)   (MGI Ref ID J:91619)
    • at 7-9 weeks of age (i.e. prior to end-stage renal disease), SCBM thickening is associated with significantly elevated matrix metalloproteinases (MMP) deposition   (MGI Ref ID J:98449)
    • treatment with an inhibitor of MMP-2, -9, -12, and -14 exacerbates SCBM thickening, directly implicating altered basement membrane metabolism in maintaining normal SCBM composition and thickness   (MGI Ref ID J:98449)

Col4a3tm1Dec/Col4a3tm1Dec

        129-Col4a3tm1Dec/J
  • mortality/aging
  • decreased survivor rate
    • impaired survival compared to Col4a3tm1Dec Sostdc1tm1Myan double homozygotes beyond 13 weeks of age   (MGI Ref ID J:158731)
  • premature death
    • impaired survival compared to Col4a3tm1Dec Sostdc1tm1Myan double homozygotes beyond 13 weeks of age   (MGI Ref ID J:158731)
  • renal/urinary system phenotype
  • abnormal renal glomerulus basement membrane morphology
    • irregular thickening and splitting of the glomerular basement membrane at 4 weeks of age by electron microscopy   (MGI Ref ID J:158731)
    • increased renal glomerulus basement membrane thickness
      • irregular thickening at 4 weeks of age   (MGI Ref ID J:158731)
  • abnormal renal glomerulus morphology
    • at 6 weeks of age, minor glomerular lesions are occasionally observed by light microscopy   (MGI Ref ID J:158731)
    • glomerulonephritis
      • progressive glomerulonephritis   (MGI Ref ID J:158731)
    • glomerulosclerosis
      • segmental sclerosis at 6 weeks of age   (MGI Ref ID J:158731)
      • at 10 weeks of age mice demonstrate glomerulosclerosis associated with inflammatory cell infiltration, interstitial fibrosis, tubular atrophy, and cast formation   (MGI Ref ID J:158731)
  • glomerulus hemorrhage
    • intraglomerular hemorrhage at 6 weeks of age   (MGI Ref ID J:158731)
  • increased urine protein level
    • at 5 weeks of age, proteinuria is initiated   (MGI Ref ID J:158731)
  • kidney failure
    • at 10 weeks of age renal function is deteriorating   (MGI Ref ID J:158731)
  • renal cast
    • renal cast formation at 10 weeks of age   (MGI Ref ID J:158731)
  • renal interstitial fibrosis
    • at 10 weeks of age, severe glomerular lesions associated with tubulointerstitial fibrosis are observed   (MGI Ref ID J:158731)
  • renal tubule atrophy
    • at 10 weeks of age mice demonstrate tubular atrophy   (MGI Ref ID J:158731)
  • homeostasis/metabolism phenotype
  • increased blood urea nitrogen level
    • at 10 weeks of age   (MGI Ref ID J:158731)
  • increased circulating creatinine level
    • at 10 weeks of age   (MGI Ref ID J:158731)
  • increased urine protein level
    • at 5 weeks of age, proteinuria is initiated   (MGI Ref ID J:158731)
  • immune system phenotype
  • glomerulonephritis
    • progressive glomerulonephritis   (MGI Ref ID J:158731)
  • cardiovascular system phenotype
  • glomerulus hemorrhage
    • intraglomerular hemorrhage at 6 weeks of age   (MGI Ref ID J:158731)

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

Col4a3tm1Dec/Col4a3tm1Dec

        involves: 129X1/SvJ * C57BL/6
  • mortality/aging
  • premature death
    • homozygotes die when end-stage renal disease develops at ~14 weeks of age   (MGI Ref ID J:37963)
  • homeostasis/metabolism phenotype
  • albuminuria
    • homozygotes develop proteinuria at ~5 weeks   (MGI Ref ID J:37963)
    • the majority of protein is of a molecular size consistent with mouse serum albumin   (MGI Ref ID J:37963)
  • hematuria
    • as early as 2 weeks, homozygotes exhibit microhematuria which persists at relatively constant levels through the course of renal disease   (MGI Ref ID J:37963)
  • increased blood urea nitrogen level
    • blood urea nitrogen levels begin to rise at ~10 weeks, and show a 10-fold increase over wild-type levels at ~14 weeks   (MGI Ref ID J:37963)
  • immune system phenotype
  • glomerulonephritis
    • homozygotes develop progressive glomerulonephritis and die with end-stage renal disease at ~14 weeks   (MGI Ref ID J:37963)
  • renal/urinary system phenotype
  • abnormal glomerular capillary morphology
    • at 8 weeks, homozygotes display thickened capillary walls with a rounded appearance   (MGI Ref ID J:37963)
    • by 14 weeks, half of the glomeruli are fibrotic with collapsed capillaries   (MGI Ref ID J:37963)
  • abnormal podocyte morphology
    • at end-stage, localized obliteration of podocytes is quite common   (MGI Ref ID J:37963)
    • podocyte foot process effacement
      • at 4 weeks, homozygotes exhibit focal swelling and obliteration of the foot processes of podocytes   (MGI Ref ID J:91619)
      • at end-stage, pedicels are effaced   (MGI Ref ID J:37963)
    • podocyte microvillus transformation
      • by 8 weeks of age, an abundance of epithelial cell microvilli are observed   (MGI Ref ID J:37963)
  • abnormal renal glomerulus basement membrane thickness
    • the mutant GBM displays focal multilaminated thickening and thinning, beginning in the external capillary loops at 4 weeks and extending throughout the GBM by 8 weeks   (MGI Ref ID J:37963)
  • albuminuria
    • homozygotes develop proteinuria at ~5 weeks   (MGI Ref ID J:37963)
    • the majority of protein is of a molecular size consistent with mouse serum albumin   (MGI Ref ID J:37963)
  • expanded mesangial matrix
    • at 4 weeks, mutants show a mild expansion of mesangial matrix in most glomeruli   (MGI Ref ID J:37963)
    • by end-stage, the mesangial matrix is grossly expanded and fibrotic   (MGI Ref ID J:37963)
  • glomerulonephritis
    • homozygotes develop progressive glomerulonephritis and die with end-stage renal disease at ~14 weeks   (MGI Ref ID J:37963)
  • granular kidney
    • at end-stage, the mutant kidney has a rough granular appearance   (MGI Ref ID J:37963)
  • hematuria
    • as early as 2 weeks, homozygotes exhibit microhematuria which persists at relatively constant levels through the course of renal disease   (MGI Ref ID J:37963)
  • mesangial cell hyperplasia
    • at 4 weeks, mutants show a mild expansion of mesangial cells in most glomeruli   (MGI Ref ID J:37963)
  • pale kidney
    • at end-stage, the mutant kidney is pale   (MGI Ref ID J:37963)
  • renal glomerulus fibrosis
    • by 14 weeks, half of the glomeruli are fibrotic   (MGI Ref ID J:37963)
  • small kidney
    • by end-stage, the mutant kidney is 30-50% smaller by mass than that of wild-type mice   (MGI Ref ID J:37963)
  • vision/eye phenotype
  • abnormal lens morphology
    • in contrast to Alport patients, homozygotes do NOT display lenticonus; however, the interior layer of the basement membrane encasing the anterior lens is irregular instead of smooth   (MGI Ref ID J:37963)
  • hearing/vestibular/ear phenotype
  • abnormal cochlear basement membrane morphology
    • COL4A5 chain is absent from all cochlear basement membranes except those in the vessels of the stria vascularis; in contrast, expression of COL4A1 and COL4A2 chains is unchanged   (MGI Ref ID J:91619)
    • homozygotes show significant thinning of the basement membrane running from the spiral limbus, down the inner sulcus, across the basilar membrane and up to the spiral prominence   (MGI Ref ID J:91619)
    • basement membranes that normally ensheathe the root cells are not detectable   (MGI Ref ID J:91619)
    • basement membranes surrounding the strial vessels are significantly thickened, with some degree of variation   (MGI Ref ID J:91619)
    • a greater abundance of COL4A1 and 4A2 chains and entactin is noted in strial basement membranes   (MGI Ref ID J:91619)
  • abnormal membranous labyrinth morphology
    • homozygotes exhibit absence of the COL4A3 and COL4A4 chains throughout the membranous labyrinth   (MGI Ref ID J:91619)
    • abnormal stria vascularis morphology
      • basement membranes surrounding the strial vessels are significantly thickened, with some degree of variation   (MGI Ref ID J:91619)
      • at 5 weeks, mutant strial basement membranes are 1.6-3.1 thicker than normal, indicating that ultrastructural changes occur prior to the onset of uremia at ~12 weeks   (MGI Ref ID J:91619)
      • a greater abundance of COL4A1 and 4A2 chains and entactin is noted in strial basement membranes   (MGI Ref ID J:91619)
      • abnormal stria vascularis vasculature morphology
        • endothelial cells lining the strial vessels are swollen and contain numerous vacuoles, resulting in capillaries with reduced internal diameters   (MGI Ref ID J:91619)
      • abnormal strial marginal cell morphology
        • homozygotes show variable changes in strial marginal cells, associated with enlarged, more rounded nuclei   (MGI Ref ID J:91619)
        • at 12-14 weeks, ~50% of mice with advanced glomerulonephritis show a reduction or loss of marginal cell basolateral infoldings   (MGI Ref ID J:91619)
  • cardiovascular system phenotype
  • abnormal glomerular capillary morphology
    • at 8 weeks, homozygotes display thickened capillary walls with a rounded appearance   (MGI Ref ID J:37963)
    • by 14 weeks, half of the glomeruli are fibrotic with collapsed capillaries   (MGI Ref ID J:37963)
  • abnormal stria vascularis vasculature morphology
    • endothelial cells lining the strial vessels are swollen and contain numerous vacuoles, resulting in capillaries with reduced internal diameters   (MGI Ref ID J:91619)
  • cellular phenotype
  • abnormal cochlear basement membrane morphology
    • COL4A5 chain is absent from all cochlear basement membranes except those in the vessels of the stria vascularis; in contrast, expression of COL4A1 and COL4A2 chains is unchanged   (MGI Ref ID J:91619)
    • homozygotes show significant thinning of the basement membrane running from the spiral limbus, down the inner sulcus, across the basilar membrane and up to the spiral prominence   (MGI Ref ID J:91619)
    • basement membranes that normally ensheathe the root cells are not detectable   (MGI Ref ID J:91619)
    • basement membranes surrounding the strial vessels are significantly thickened, with some degree of variation   (MGI Ref ID J:91619)
    • a greater abundance of COL4A1 and 4A2 chains and entactin is noted in strial basement membranes   (MGI Ref ID J:91619)

Col4a3tm1Dec/Col4a3tm1Dec

        B6.129X1-Col4a3tm1Dec
  • mortality/aging
  • premature death
    • mice die between 23 and 30 weeks   (MGI Ref ID J:167404)
  • renal/urinary system phenotype
  • abnormal renal glomerulus basement membrane thickness
    • at 22 weeks, the glomerular basement membrane exhibits focal thinning and thickening compared to in wild-type mice   (MGI Ref ID J:167404)
  • glomerulonephritis
    • beginning at 12 weeks and extensive at 22 weeks   (MGI Ref ID J:167404)
  • increased urine protein level
  • podocyte foot process effacement
  • renal interstitial fibrosis
    • beginning at 12 weeks and extensive at 22 weeks   (MGI Ref ID J:167404)
  • renal tubule atrophy
    • beginning at 12 weeks and extensive at 22 weeks, mice exhibit tubular atrophy unlike wild-type mice   (MGI Ref ID J:167404)
  • homeostasis/metabolism phenotype
  • increased circulating creatinine level
  • increased urine protein level
  • immune system phenotype
  • glomerulonephritis
    • beginning at 12 weeks and extensive at 22 weeks   (MGI Ref ID J:167404)
View Research Applications

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

Col4a3tm1Dec related

Developmental Biology Research
Defects in Extracellular Matrix Molecules
Internal/Organ Defects

Neurobiology Research
Hearing Defects

Sensorineural Research
Hearing Defects

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Col4a3tm1Dec
Allele Name targeted mutation 1, Dominic Cosgrove
Allele Type Targeted (Null/Knockout)
Mutation Made ByDr. Dominic Cosgrove,   Boys Town National Research Hospital
Strain of Origin129X1/SvJ
Gene Symbol and Name Col4a3, collagen, type IV, alpha 3
Chromosome 1
Gene Common Name(s) alpha3(IV); tumstatin;
Molecular Note A neomycin selection cassette was inserted into exon 5. Northern blot analysis on RNA derived from kidney of homozygous mice demonstrated that no detectable transcript is produced from this allele. Immunohistochemistry experiments on kidney sections from homozygous mice confirmed that no detectable protein is made. [MGI Ref ID J:37963]

Genotyping

Genotyping Information

Genotyping Protocols

Col4a3tm1Decalternate1,

Separated MCA


Col4a3tm1Decalternate1, Separated PCR
NEOTD (Generic Neo), Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Cosgrove D; Meehan DT; Grunkemeyer JA; Kornak JM; Sayers R; Hunter WJ; Samuelson GC. 1996. Collagen COL4A3 knockout: a mouse model for autosomal Alport syndrome. Genes Dev 10(23):2981-92. [PubMed: 8956999]  [MGI Ref ID J:37963]

Additional References

Cosgrove D; Rodgers K; Meehan D; Miller C; Bovard K; Gilroy A; Gardner H; Kotelianski V; Gotwals P; Amatucci A; Kalluri R. 2000. Integrin alpha1beta1 and transforming growth factor-beta1 play distinct roles in alport glomerular pathogenesis and serve as dual targets for metabolic therapy. Am J Pathol 157(5):1649-59. [PubMed: 11073824]  [MGI Ref ID J:91620]

Cosgrove D; Samuelson G; Meehan DT; Miller C; McGee J; Walsh EJ; Siegel M. 1998. Ultrastructural, physiological, and molecular defects in the inner ear of a gene-knockout mouse model for autosomal Alport syndrome. Hear Res 121(1-2):84-98. [PubMed: 9682811]  [MGI Ref ID J:91619]

Miner JH; Sanes JR. 1994. Collagen IV alpha 3, alpha 4, and alpha 5 chains in rodent basal laminae: sequence, distribution, association with laminins, and developmental switches. J Cell Biol 127(3):879-91. [PubMed: 7962065]  [MGI Ref ID J:21122]

Col4a3tm1Dec related

Abrahamson DR; Isom K; Roach E; Stroganova L; Zelenchuk A; Miner JH; St John PL. 2007. Laminin compensation in collagen alpha3(IV) knockout (Alport) glomeruli contributes to permeability defects. J Am Soc Nephrol 18(9):2465-72. [PubMed: 17699809]  [MGI Ref ID J:148663]

Abrahamson DR; Prettyman AC; Robert B; St John PL. 2003. Laminin-1 reexpression in Alport mouse glomerular basement membranes. Kidney Int 63(3):826-34. [PubMed: 12631063]  [MGI Ref ID J:103978]

Baxter RM; Crowell TP; George JA; Getman ME; Gardner H. 2007. The plant pathogenesis related protein GLIPR-2 is highly expressed in fibrotic kidney and promotes epithelial to mesenchymal transition in vitro. Matrix Biol 26(1):20-9. [PubMed: 17055234]  [MGI Ref ID J:117359]

Beirowski B; Weber M; Gross O. 2006. Chronic renal failure and shortened lifespan in COL4A3+/- mice: an animal model for thin basement membrane nephropathy. J Am Soc Nephrol 17(7):1986-94. [PubMed: 16775036]  [MGI Ref ID J:128226]

Clauss S; Gross O; Kulkarni O; Avila-Ferrufino A; Radomska E; Segerer S; Eulberg D; Klussmann S; Anders HJ. 2008. Ccl2/Mcp-1 blockade reduces glomerular and interstitial macrophages but does not ameliorate renal pathology in collagen4A3-deficient mice with autosomal recessive Alport nephropathy. J Pathol :. [PubMed: 19156777]  [MGI Ref ID J:146525]

Cosgrove D; Kalluri R; Miner JH; Segal Y; Borza DB. 2007. Choosing a mouse model to study the molecular pathobiology of Alport glomerulonephritis. Kidney Int 71(7):615-8. [PubMed: 17290292]  [MGI Ref ID J:188471]

Cosgrove D; Meehan DT; Delimont D; Pozzi A; Chen X; Rodgers KD; Tempero RM; Zallocchi M; Rao VH. 2008. Integrin {alpha}1{beta}1 Regulates Matrix Metalloproteinases via P38 Mitogen-Activated Protein Kinase in Mesangial Cells: Implications for Alport Syndrome. Am J Pathol 172(3):761-73. [PubMed: 18258846]  [MGI Ref ID J:132013]

Cosgrove D; Rodgers K; Meehan D; Miller C; Bovard K; Gilroy A; Gardner H; Kotelianski V; Gotwals P; Amatucci A; Kalluri R. 2000. Integrin alpha1beta1 and transforming growth factor-beta1 play distinct roles in alport glomerular pathogenesis and serve as dual targets for metabolic therapy. Am J Pathol 157(5):1649-59. [PubMed: 11073824]  [MGI Ref ID J:91620]

Cosgrove D; Samuelson G; Meehan DT; Miller C; McGee J; Walsh EJ; Siegel M. 1998. Ultrastructural, physiological, and molecular defects in the inner ear of a gene-knockout mouse model for autosomal Alport syndrome. Hear Res 121(1-2):84-98. [PubMed: 9682811]  [MGI Ref ID J:91619]

Dai B; David V; Alshayeb HM; Showkat A; Gyamlani G; Horst RL; Wall BM; Quarles LD. 2012. Assessment of 24,25(OH)2D levels does not support FGF23-mediated catabolism of vitamin D metabolites. Kidney Int 82(10):1061-70. [PubMed: 22739976]  [MGI Ref ID J:198168]

David V; Dai B; Martin A; Huang J; Han X; Quarles LD. 2013. Calcium regulates FGF-23 expression in bone. Endocrinology 154(12):4469-82. [PubMed: 24140714]  [MGI Ref ID J:206528]

Dennis J; Meehan DT; Delimont D; Zallocchi M; Perry GA; O'Brien S; Tu H; Pihlajaniemi T; Cosgrove D. 2010. Collagen XIII induced in vascular endothelium mediates alpha1beta1 integrin-dependent transmigration of monocytes in renal fibrosis. Am J Pathol 177(5):2527-40. [PubMed: 20864678]  [MGI Ref ID J:166258]

Gratton MA; Rao VH; Meehan DT; Askew C; Cosgrove D. 2005. Matrix metalloproteinase dysregulation in the stria vascularis of mice with alport syndrome: implications for capillary basement membrane pathology. Am J Pathol 166(5):1465-74. [PubMed: 15855646]  [MGI Ref ID J:98449]

Gross O; Beirowski B; Koepke ML; Kuck J; Reiner M; Addicks K; Smyth N; Schulze-Lohoff E; Weber M. 2003. Preemptive ramipril therapy delays renal failure and reduces renal fibrosis in COL4A3-knockout mice with Alport syndrome. Kidney Int 63(2):438-46. [PubMed: 12631109]  [MGI Ref ID J:129179]

Gross O; Girgert R; Beirowski B; Kretzler M; Kang HG; Kruegel J; Miosge N; Busse AC; Segerer S; Vogel WF; Muller GA; Weber M. 2010. Loss of collagen-receptor DDR1 delays renal fibrosis in hereditary type IV collagen disease. Matrix Biol 29(5):346-56. [PubMed: 20307660]  [MGI Ref ID J:161975]

Hahm K; Lukashev ME; Luo Y; Yang WJ; Dolinski BM; Weinreb PH; Simon KJ; Chun Wang L; Leone DR; Lobb RR; McCrann DJ; Allaire NE; Horan GS; Fogo A; Kalluri R; Shield CF 3rd; Sheppard D; Gardner HA; Violette SM. 2007. Alpha v beta6 integrin regulates renal fibrosis and inflammation in Alport mouse. Am J Pathol 170(1):110-25. [PubMed: 17200187]  [MGI Ref ID J:117045]

Hamano Y; Grunkemeyer JA; Sudhakar A; Zeisberg M; Cosgrove D; Morello R; Lee B; Sugimoto H; Kalluri R. 2002. Determinants of vascular permeability in the kidney glomerulus. J Biol Chem 277(34):31154-62. [PubMed: 12039968]  [MGI Ref ID J:78616]

Hamano Y; Zeisberg M; Sugimoto H; Lively JC; Maeshima Y; Yang C; Hynes RO; Werb Z; Sudhakar A; Kalluri R. 2003. Physiological levels of tumstatin, a fragment of collagen IV alpha3 chain, are generated by MMP-9 proteolysis and suppress angiogenesis via alphaV beta3 integrin. Cancer Cell 3(6):589-601. [PubMed: 12842087]  [MGI Ref ID J:120382]

Heidet L; Borza DB; Jouin M; Sich M; Mattei MG; Sado Y; Hudson BG; Hastie N; Antignac C; Gubler MC. 2003. A human-mouse chimera of the alpha3alpha4alpha5(IV) collagen protomer rescues the renal phenotype in Col4a3-/- Alport mice. Am J Pathol 163(4):1633-44. [PubMed: 14507670]  [MGI Ref ID J:109362]

Kalluri R; Cosgrove D. 2000. Assembly of type IV collagen. Insights from alpha3(IV) collagen-deficient mice. J Biol Chem 275(17):12719-24. [PubMed: 10777566]  [MGI Ref ID J:120234]

Kang JS; Wang XP; Miner JH; Morello R; Sado Y; Abrahamson DR; Borza DB. 2006. Loss of alpha3/alpha4(IV) collagen from the glomerular basement membrane induces a strain-dependent isoform switch to alpha5alpha6(IV) collagen associated with longer renal survival in Col4a3-/- Alport mice. J Am Soc Nephrol 17(7):1962-9. [PubMed: 16769745]  [MGI Ref ID J:128227]

LeBleu V; Sund M; Sugimoto H; Birrane G; Kanasaki K; Finan E; Miller CA; Gattone VH 2nd; McLaughlin H; Shield CF 3rd; Kalluri R. 2010. Identification of the NC1 domain of {alpha}3 chain as critical for {alpha}3{alpha}4{alpha}5 type IV collagen network assembly. J Biol Chem 285(53):41874-85. [PubMed: 20847057]  [MGI Ref ID J:167404]

Lebleu VS; Sugimoto H; Miller CA; Gattone VH 2nd; Kalluri R. 2008. Lymphocytes are dispensable for glomerulonephritis but required for renal interstitial fibrosis in matrix defect-induced Alport renal disease. Lab Invest 88(3):284-92. [PubMed: 18180701]  [MGI Ref ID J:131825]

Lebleu VS; Taduri G; O'Connell J; Teng Y; Cooke VG; Woda C; Sugimoto H; Kalluri R. 2013. Origin and function of myofibroblasts in kidney fibrosis. Nat Med 19(8):1047-53. [PubMed: 23817022]  [MGI Ref ID J:200001]

Ninichuk V; Gross O; Segerer S; Hoffmann R; Radomska E; Buchstaller A; Huss R; Akis N; Schlondorff D; Anders HJ. 2006. Multipotent mesenchymal stem cells reduce interstitial fibrosis but do not delay progression of chronic kidney disease in collagen4A3-deficient mice. Kidney Int 70(1):121-9. [PubMed: 16723981]  [MGI Ref ID J:188472]

Rao VH; Meehan DT; Delimont D; Nakajima M; Wada T; Gratton MA; Cosgrove D. 2006. Role for macrophage metalloelastase in glomerular basement membrane damage associated with alport syndrome. Am J Pathol 169(1):32-46. [PubMed: 16816359]  [MGI Ref ID J:110183]

Ryu M; Kulkarni OP; Radomska E; Miosge N; Gross O; Anders HJ. 2011. Bacterial CpG-DNA accelerates Alport glomerulosclerosis by inducing an M1 macrophage phenotype and tumor necrosis factor-alpha-mediated podocyte loss. Kidney Int 79(2):189-98. [PubMed: 20962742]  [MGI Ref ID J:186887]

Ryu M; Migliorini A; Miosge N; Gross O; Shankland S; Brinkkoetter PT; Hagmann H; Romagnani P; Liapis H; Anders HJ. 2012. Plasma leakage through glomerular basement membrane ruptures triggers the proliferation of parietal epithelial cells and crescent formation in non-inflammatory glomerular injury. J Pathol :. [PubMed: 22553158]  [MGI Ref ID J:189436]

Sampson NS; Ryan ST; Enke DA; Cosgrove D; Koteliansky V; Gotwals P. 2001. Global gene expression analysis reveals a role for the alpha 1 integrin in renal pathogenesis. J Biol Chem 276(36):34182-8. [PubMed: 11447218]  [MGI Ref ID J:71378]

Sayers R; Kalluri R; Rodgers KD; Shield CF; Meehan DT; Cosgrove D. 1999. Role for transforming growth factor-beta1 in alport renal disease progression. Kidney Int 56(5):1662-73. [PubMed: 10571774]  [MGI Ref ID J:59648]

Steenhard BM; Vanacore R; Friedman D; Zelenchuk A; Stroganova L; Isom K; St John PL; Hudson BG; Abrahamson DR. 2012. Upregulated expression of integrin alpha1 in mesangial cells and integrin alpha3 and vimentin in podocytes of Col4a3-null (Alport) mice. PLoS One 7(12):e50745. [PubMed: 23236390]  [MGI Ref ID J:195680]

Sund M; Hamano Y; Sugimoto H; Sudhakar A; Soubasakos M; Yerramalla U; Benjamin LE; Lawler J; Kieran M; Shah A; Kalluri R. 2005. Function of endogenous inhibitors of angiogenesis as endothelium-specific tumor suppressors. Proc Natl Acad Sci U S A 102(8):2934-9. [PubMed: 15710885]  [MGI Ref ID J:97878]

Tanaka M; Asada M; Higashi AY; Nakamura J; Oguchi A; Tomita M; Yamada S; Asada N; Takase M; Okuda T; Kawachi H; Economides AN; Robertson E; Takahashi S; Sakurai T; Goldschmeding R; Muso E; Fukatsu A; Kita T; Yanagita M. 2010. Loss of the BMP antagonist USAG-1 ameliorates disease in a mouse model of the progressive hereditary kidney disease Alport syndrome. J Clin Invest 120(3):768-77. [PubMed: 20197625]  [MGI Ref ID J:158731]

Touchberry CD; Green TM; Tchikrizov V; Mannix JE; Mao TF; Carney BW; Girgis M; Vincent RJ; Wetmore LA; Dawn B; Bonewald LF; Stubbs JR; Wacker MJ. 2013. FGF23 is a novel regulator of intracellular calcium and cardiac contractility in addition to cardiac hypertrophy. Am J Physiol Endocrinol Metab 304(8):E863-73. [PubMed: 23443925]  [MGI Ref ID J:196066]

Xie L; Duncan MB; Pahler J; Sugimoto H; Martino M; Lively J; Mundel T; Soubasakos M; Rubin K; Takeda T; Inoue M; Lawler J; Hynes RO; Hanahan D; Kalluri R. 2011. Counterbalancing angiogenic regulatory factors control the rate of cancer progression and survival in a stage-specific manner. Proc Natl Acad Sci U S A 108(24):9939-44. [PubMed: 21622854]  [MGI Ref ID J:173322]

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* $2525.00
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.

Frozen Products

Price (US dollars $)
Frozen Embryo $1650.00

Standard Supply

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

Supply Notes

  • Cryopreserved Embryos
    Available to most shipping destinations1
    This strain is also available as cryopreserved embryos2. Orders for cryopreserved embryos may be placed with our Customer Service Department. Experienced technicians at The Jackson Laboratory have recovered frozen embryos of this strain successfully. We will provide you enough embryos to perform two embryo transfers. The Jackson Laboratory does not guarantee successful recovery at your facility. For complete information on purchasing embryos, please visit our Cryopreserved Embryos web page.

    1 Shipments cannot be made to Australia due to Australian government import restrictions.
    2 Embryos for most strains are cryopreserved at the two cell stage while some strains are cryopreserved at the eight cell stage. If this information is important to you, please contact Customer Service.
  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We willfulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. 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* $3283.00
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.

Frozen Products

Price (US dollars $)
Frozen Embryo $2145.00

Standard Supply

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

Supply Notes

  • Cryopreserved Embryos
    Available to most shipping destinations1
    This strain is also available as cryopreserved embryos2. Orders for cryopreserved embryos may be placed with our Customer Service Department. Experienced technicians at The Jackson Laboratory have recovered frozen embryos of this strain successfully. We will provide you enough embryos to perform two embryo transfers. The Jackson Laboratory does not guarantee successful recovery at your facility. For complete information on purchasing embryos, please visit our Cryopreserved Embryos web page.

    1 Shipments cannot be made to Australia due to Australian government import restrictions.
    2 Embryos for most strains are cryopreserved at the two cell stage while some strains are cryopreserved at the eight cell stage. If this information is important to you, please contact Customer Service.
  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We willfulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. 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.

Control Information

  Control
   Wild-type from the colony
   000691 129X1/SvJ
 
  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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