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Former Names BUB/BnJ-Pde6brd1 (Changed: 19-MAR-08 ) Type Inbred Strain; Additional information on Inbred Strains. Visit our online Nomenclature tutorial. Mating System Sibling x Sibling (Female x Male) 01-MAR-06 Species laboratory mouse H2 Haplotype q2 (see, Fischer Lindahl K 1997 and Shen FW 1982) Generation F200 (05-FEB-09) Appearance
albino
Related Genotype: a/a Tyrc/TyrcImportant Note
This strain is homozygous for Cdh23ahl and Pde6brd1. The ahl mutation accounts for progressive hearing loss (complete deafness by two to three months). See article "Genetic Background Effects: Can Your Mice See?", JAX® NOTES Spring 2002, No. 485.Description
BUB/BnJ mice carry a specific T cell receptor V beta mutation, making this strain highly susceptible to collagen-induced arthritis. This T cell receptor V beta restriction works in concert with other uncharacterized modifying genes present in BUB/BnJ mice. BUB/BnJ mice carry no detectable endogenous ecotropic MuLV DNA sequences. Mice are also reported to have high serum complement activity. In response to challenge, BUB/BnJ mice develop immune-mediated nephritis characterized by proteinuria, glomerulonephritis and tubulointerstitial disease (Xie et al., 2004).BUB/BnJ mice are homozygous for the Mass1frings allele (monogenic, audiogenic seizure susceptibility 1) and are susceptible to audiogenic seizures prior to 25 days of age (Skradski, et al 2001). In addition, the Mass1frings allele acounts for early onset hearing impairment by 3-4 weeks of age (Johnson KR, et al 2005).
Development
The BUB/BnJ inbred strain originated from randomly bred albino mice of unknown ancestry. Inbreeding was begun in 1945 by JW Wilson at Brown University. This strain was transferred to S Bernstein at The Jackson Laboratory at F46 and then to the production facility in 1968 at generation F68. Current generation of inbreeding is F166+.
Strains carrying Cdh23ahl allele
001137 129P1/ReJ 000690 129P3/J 000691 129X1/SvJ 000646 A/J 000647 A/WySnJ 003070 ALR/LtJ 003072 ALS/LtJ 004502 B6;AKR-Lxl2/GrsrJ 001026 BALB/cByJ 005494 C3.129S1(B6)-Grm1rcw/J 000664 C57BL/6J 004764 C57BL/6J-Cdh23v-8J/J 003129 C57BL/6J-Epha4rb-2J/GrsrJ 004820 C57BL/6J-Kcne12J/J 004703 C57BL/6J-Kcnq2Nmf134/J 004811 C57BL/6J-nmf110/J 004812 C57BL/6J-nmf111/J 004747 C57BL/6J-nmf118/J 004656 C57BL/6J-nmf88/J 004391 C57BL/6J-Chr 13A/J/NaJ 004385 C57BL/6J-Chr 7A/J/NaJ 000662 C57BLKS/J 000667 C57BR/cdJ 000668 C57L/J 000669 C58/J 000657 CE/J 000670 DBA/1J 001140 DBA/1LacJ 000671 DBA/2J 007048 DBA/2J-Gpnmb+/SjJ 002106 KK/HlJ 000675 LG/J 000676 LP/J 000677 MA/MyJ 001976 NOD/ShiLtJ 002050 NOR/LtJ 000679 P/J 002747 SENCARB/PtJ 002335 SKH2/J 003392 STOCK Crb1rd8/J View Strains carrying Cdh23ahl (40 strains)
Strains carrying Il3ram1 allele
000645 A/HeJ 000646 A/J 000647 A/WySnJ 000648 AKR/J 000669 C58/J 000657 CE/J 000684 NZB/BlNJ 000682 RF/J 000687 SM/J View Strains carrying Il3ram1 (9 strains)
Strains carrying Pde6brd1 allele
View Strains carrying Pde6brd1 (74 strains)
Strains carrying other alleles of Cdh23
002756 B6.CAST-Cdh23Ahl+/Kjn 002432 B6J x B6.C-H2-Kbm1/ByJ-Cdh23v-J/J 002552 C57BL/6J-Cdh23v-2J/J 004764 C57BL/6J-Cdh23v-8J/J 004819 C57BL/6J-Cdh23v-9J/J 005016 CByJ;B6-Cdh23v-10J/J 000275 V/LeJ View Strains carrying other alleles of Cdh23 (7 strains)
Strains carrying other alleles of Pde6b
004297 B6.CXB1-Pde6brd10/J 003647 B6EiC3Sn.BLiAF1 002802 C3.BLiA Pde6b+-Krd/J 001979 C3A.BLiA-Pde6b+.O20-Prph2Rd2/J 001912 C3A.BLiA-Pde6b+/J 003648 C3Sn.BLiA-Pde6b+/Dn 004766 C57BL/6J-Pde6brd1-2J/J 004828 FVB.129P2-Pde6b+ Tyrc-ch/AntJ 004808 STOCK Mapttm1(EGFP)Klt Tg(MAPT)8cPdav/J View Strains carrying other alleles of Pde6b (9 strains)
Genetic Quality Control Annual Report
JAX® NOTES, April 1988; 433. H-2 Haplotypes of Mice from Jackson Laboratory Production Colonies.
JAX® NOTES, Spring 2002; 485. Genetic Background Effects: Can Your Mice See?
View Phenotypic Data
Phenotypic Data
Mouse Phenome Database
Festing Inbred Strain Characteristics: BUB
View Mammalian Phenotype Terms
Mammalian Phenotype Terms
assigned by genotype
Il3ram1/Il3ram1
BUB/BnJ
- hematopoietic system phenotype
- abnormal common myeloid progenitor cell morphology (MGI Ref ID J:24918)
- CFU-GM assays using bone marrow derived cells yield very few colonies in repsonse to interleukin 3
View Research Applications
Research Applications
This mouse can be used to support research in many areas including:
Cdh23ahl relatedImmunology and Inflammation Research
Inflammation
collagen induced arthritis
Neurobiology Research
Epilepsy
audiogenic seizures
Vestibular and Hearing Defects
Age related hearing loss
Research Tools
Cancer Research
no detectable endogenous ecotropic MuLV DNA Sequences
Sensorineural Research
Retinal Degeneration
Homozygous for Pde6brd1
Vestibular and Hearing Defects
Age related hearing loss
Il3ram1 relatedNeurobiology Research
Vestibular and Hearing Defects
Age related hearing loss
Sensorineural Research
Vestibular and Hearing Defects
Age related hearing loss
Pde6brd1 relatedImmunology and Inflammation Research
CD Antigens, Antigen Receptors, and Histocompatibility Markers
genes regulating susceptibility to infectious disease and endotoxin
Mouse/Human Gene Homologs
retinitis pigmentosa, autosomal recessive
Sensorineural Research
Retinal Degeneration
| Allele Symbol | Cdh23ahl | ||
|---|---|---|---|
| Allele Name | age related hearing loss 1 | ||
| Allele Type | QTL | ||
| Common Name(s) | Cdh23753A; mdfw; | ||
| Strain of Origin | multiple strains | ||
| Gene Symbol and Name | Cdh23, cadherin 23 (otocadherin) | ||
| Chromosome | 10 | ||
| Gene Common Name(s) | 4930542A03Rik; DFNB12; DKFZp434P2350; FLJ00233; FLJ36499; KIAA1774; KIAA1812; MGC102761; RIKEN cDNA 4930542A03 gene; USH1D; W; age related hearing loss 1; ahl; bob; bobby; bus; bustling; mdfw; modifier of deaf waddler; neuroscience mutagenesis facility, 112; neuroscience mutagenesis facility, 181; neuroscience mutagenesis facility, 252; nmf112; nmf181; nmf252; sals; salsa; v; waltzer; | ||
| Molecular Note | Genetic complementation tests have shown allelism between the mdfw (modifier of deaf waddler) locus and the ahl locus. Further analysis has identified an association between ahl and a G to A transition at nucleotide position 753 of Cdh23. This hypomorphic allele causes in frame skipping of exon 7 and reduced message stability. Twenty-seven strains classified with ahl and carrying the 753A allele include: CD1, RBF/DnJ, PL/J, AKR/J, RF/J, BALB/cBy, A/WySnJ, P/J, SENCARA/PtJ, DBA/1J, ALS/LtJ, C58/J, C57BLKS/J, 129P1/ReJ, C57BR/cd, SKH2/J, BUB/Bn, MA/MyJ, LP/J, 129X1/SvJ, NOR/LtJ, A/J, C57BL/6, NOD/LtJ, DBA/2J, ALR/LtJ, C57L/J. Strains classified with ahl that DO NOT carry this mutation include: C3H/HeSnJ, I/LnJ,YBR/Ei, MRL/MpJ. [MGI Ref ID J:86905] | ||
| Allele Symbol | Il3ram1 | ||
| Allele Name | mutation 1 | ||
| Allele Type | Spontaneous | ||
| Common Name(s) | Il3raA/J; Il3ran; | ||
| Strain of Origin | A/J | ||
| Gene Symbol and Name | Il3ra, interleukin 3 receptor, alpha chain | ||
| Chromosome | 14 | ||
| Gene Common Name(s) | CD123; CDw123; Cyrl; IL-3 receptor alpha chain; IL3R; IL3RAY; IL3RX; IL3RY; Il-3 alpha subunit; MGC34174; SUT-1; hIL-3Ra; | ||
| Molecular Note | Sequence analysis revealed A/J mice lack the sequence corresponding to exon 8, which encodes 10 amino acid residues in the extracellular domain. Aberrant splicing was due to a 5 base pair deletion at the branch point in intron 7. [MGI Ref ID J:23971] | ||
| Allele Symbol | Pde6brd1 | ||
| Allele Name | retinal degeneration 1 | ||
| Allele Type | Spontaneous | ||
| Common Name(s) | Pdebrd1; rd; rd-1; rd1; rodless retina; | ||
| Gene Symbol and Name | Pde6b, phosphodiesterase 6B, cGMP, rod receptor, beta polypeptide | ||
| Chromosome | 5 | ||
| Gene Common Name(s) | CSNB3; PDEB; Pdeb; RP40; nmf137; phosphodiesterase, cGMP, rod receptor, beta polypeptide; r; rd; rd-1; rd1; rd10; retinal degeneration; retinal degeneration 1; retinal degeneration 10; | ||
| General Note | CBA/J mice carry this allele. | ||
| Molecular Note | Two mutations have been identified in rd1 mice. A murine leukimia virus (Xmv-28) insertion in reverse orientation in intron 1 is found in all mouse strains with the rd1 phenotype. Further, a nonsense mutation (C to A transversion) in codon 347 that results in a truncation eliminating more than half of the predicted encoded protein, including the catalytic domain has also been identified in all rd1 strains of mice. A specific degradation of mutant transcript during or after pre-mRNA splicing is suggested. [MGI Ref ID J:11513] [MGI Ref ID J:4366] [MGI Ref ID J:51361] | ||
This strain will not have a genotyping protocol or one is not currently available.
Helpful Links
Genotyping resources and troubleshooting
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Shen FW; Chorney MJ; Boyse EA. 1982. Further polymorphism of the Tla locus defined by monoclonal TL antibodies. Immunogenetics 15(6):573-8. [PubMed: 7106865] [MGI Ref ID J:6828]
Haqqi TM; Qu XM; Sy MS; Banerjee S. 1995. Restricted expression of T cell receptor V beta and lymphokine genes in arthritic joints of a TCR V beta a (H-2q) mouse strain-BUB/BnJ-with collagen-induced arthritis. Autoimmunity 20(3):163-70. [PubMed: 7578877] [MGI Ref ID J:28621]
Hara T; Ichihara M; Takagi M; Miyajima A. 1995. Interleukin-3 (IL-3) poor-responsive inbred mouse strains carry the identical deletion of a branch point in the IL-3 receptor alpha subunit gene. Blood 85(9):2331-6. [PubMed: 7727767] [MGI Ref ID J:24918]
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Ortman RA; Holderbaum D; Qu XM; Banerjee S; Haqqi TM. 1994. BUB/BnJ (H-2q) is a TCR deletion mutant mouse strain (TCR V beta a, KJ16-) that is susceptible to type II collagen-induced arthritis. J Immunol 152(8):4175-82. [PubMed: 8144978] [MGI Ref ID J:17601]
Xie C; Sharma R; Wang H; Zhou XJ; Mohan C. 2004. Strain distribution pattern of susceptibility to immune-mediated nephritis. J Immunol 172(8):5047-55. [PubMed: 15067087] [MGI Ref ID J:122988]
Cdh23ahl relatedIl3ram1 relatedDavis RR; Newlander JK; Ling X; Cortopassi GA; Krieg EF; Erway LC. 2001. Genetic basis for susceptibility to noise-induced hearing loss in mice. Hear Res 155(1-2):82-90. [PubMed: 11335078] [MGI Ref ID J:69679]
Di Palma F; Pellegrino R; Noben-Trauth K. 2001. Genomic structure, alternative splice forms and normal and mutant alleles of cadherin 23 (Cdh23). Gene 281(1-2):31-41. [PubMed: 11750125] [MGI Ref ID J:73941]
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Johnson KR; Longo-Guess C; Gagnon LH; Yu H; Zheng QY. 2008. A locus on distal chromosome 11 (ahl8) and its interaction with Cdh23 ahl underlie the early onset, age-related hearing loss of DBA/2J mice. Genomics 92(4):219-25. [PubMed: 18662770] [MGI Ref ID J:139223]
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Johnson KR; Zheng QY; Weston MD; Ptacek LJ; Noben-Trauth K. 2005. The Mass1(frings) mutation underlies early onset hearing impairment in BUB/BnJ mice, a model for the auditory pathology of Usher syndrome IIC. Genomics 85(5):582-90. [PubMed: 15820310] [MGI Ref ID J:97534]
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Mathews CE; Leiter EH. 1999. Resistance of ALR/Lt islets to free radical-mediated diabetogenic stress is inherited as a dominant trait. Diabetes 48(11):2189-96. [PubMed: 10535453] [MGI Ref ID J:109893]
Nadeau JH. 2003. Modifier genes and protective alleles in humans and mice. Curr Opin Genet Dev 13(3):290-5. [PubMed: 12787792] [MGI Ref ID J:88012]
Noben-Trauth K; Zheng QY; Johnson KR. 2003. Association of cadherin 23 with polygenic inheritance and genetic modification of sensorineural hearing loss. Nat Genet 35(1):21-3. [PubMed: 12910270] [MGI Ref ID J:86905]
Noben-Trauth K; Zheng QY; Johnson KR; Nishina PM. 1997. mdfw: a deafness susceptibility locus that interacts with deaf waddler (dfw). Genomics 44(3):266-72. [PubMed: 9325047] [MGI Ref ID J:38429]
Vazquez AE; Jimenez AM; Martin GK; Luebke AE; Lonsbury-Martin BL. 2004. Evaluating cochlear function and the effects of noise exposure in the B6.CAST+Ahl mouse with distortion product otoacoustic emissions. Hear Res 194(1-2):87-96. [PubMed: 15276680] [MGI Ref ID J:117746]
Zheng QY; Johnson KR. 2001. Hearing loss associated with the modifier of deaf waddler (mdfw) locus corresponds with age-related hearing loss in 12 inbred strains of mice. Hear Res 154(1-2):45-53. [PubMed: 11423214] [MGI Ref ID J:70964]
Pde6brd1 relatedGainsford T; Roberts AW; Kimura S; Metcalf D; Dranoff G; Mulligan RC; Begley CG; Robb L; Alexander WS. 1998. Cytokine production and function in c-mpl-deficient mice: no physiologic role for interleukin-3 in residual megakaryocyte and platelet production. Blood 91(8):2745-52. [PubMed: 9531584] [MGI Ref ID J:47462]
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Ichihara M; Hara T; Takagi M; Cho LC; Gorman DM; Miyajima A. 1995. Impaired interleukin-3 (IL-3) response of the A/J mouse is caused by a branch point deletion in the IL-3 receptor alpha subunit gene. EMBO J 14(5):939-50. [PubMed: 7889941] [MGI Ref ID J:23971]
Acosta ML; Fletcher EL; Azizoglu S; Foster LE; Farber DB; Kalloniatis M. 2005. Early markers of retinal degeneration in rd/rd mice. Mol Vis 11:717-28. [PubMed: 16163270] [MGI Ref ID J:103970]
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Ahuja S; Ahuja-Jensen P; Johnson LE; Caffe AR; Abrahamson M; Ekstrom PA; van Veen T. 2008. rd1 Mouse retina shows an imbalance in the activity of cysteine protease cathepsins and their endogenous inhibitor cystatin C. Invest Ophthalmol Vis Sci 49(3):1089-96. [PubMed: 18326735] [MGI Ref ID J:133024]
Ahuja-Jensen P; Johnsen-Soriano S; Ahuja S; Bosch-Morell F; Sancho-Tello M; Romero FJ; Abrahamson M; van Veen T. 2007. Low glutathione peroxidase in rd1 mouse retina increases oxidative stress and proteases. Neuroreport 18(8):797-801. [PubMed: 17471069] [MGI Ref ID J:122802]
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Animal Health Reports
Room Number AX11
Colony Maintenance
Mating System Sibling x Sibling (Female x Male) 01-MAR-06 Diet Information LabDiet® 5K52/5K67
| Pricing for USA, Canada and Mexico shipping destinations |
|
Weeks of Age Price (US dollars $) Gender Individual Mouse $56.30 Female or Male
| Pricing for International shipping destinations |
|
Weeks of Age Price (US dollars $) Gender Individual Mouse $73.20 Female or Male
| Standard Supply | Repository-Live. A collection of over 1000 strains maintained as live colonies. Individual colonies are sized to meet current customer demand. Delivery for orders of 10 mice or less ranges on average from one to eight weeks; mice are generally shipped between four to six weeks of age with a maximum shipping age of approximately nine weeks. Colony sizes do not generally support stringent age specifications for large volumes of mice; however custom orders and larger quantities of mice are easily arranged. Estimated ship dates for all orders provided within two business days following order placement. |
|---|---|
| Supply Notes |
|
| Important Note | |
| This strain is homozygous for Cdh23ahl and Pde6brd1. The ahl mutation accounts for progressive hearing loss (complete deafness by two to three months). See article "Genetic Background Effects: Can Your Mice See?", JAX® NOTES Spring 2002, No. 485. | |
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| phone: | 207-288-6470 |
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