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- Description
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- Genes & alleles
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Description
Strain Information
Former Names C57BL/6J-Chr 13A/NaJ (Changed: 19-SEP-06 ) C57BL/6J-13A/Na (Changed: 15-DEC-04 ) Type Consomic; Additional information on Consomic Mice. Visit our online Nomenclature tutorial. Mating System Sibling x Sibling (Female x Male) 01-MAR-06 Species laboratory mouse Background Strain C57BL/6J Donor Strain A/J H2 Haplotype b Generation N13F4+22 (21-DEC-10)
Generation DefinitionsAppearance
black
Related Genotype: a/aImportant Note
The C57BL/6J background strain is homozygous for the age related hearing loss mutation Cdh23ahl, which on this background causes progressive hearing loss with onset after 10 months of age.Description
A chromosome substitution or consomic strain is an inbred strain with one of its chromosomes replaced by the homologous chromosome of another inbred strain. The C57BL/6J and A/J strains in this set were chosen because they differ in their susceptibility to diseases such as arthritis, asthma, atherosclerosis, cancer, several infectious diseases, inflammatory responses, and physiological, behavioral and sensory phenotypes. Chromosome substitution strain nomenclature is designated as Host Strain - Chromosome #<Donor Strain>/Laboratory code. For example, C57BL/6J-Chr1A/NaJ carries chromosome 1 for strain A/J (A) on a C57BL/6J background, was constructed in the laboratory of Joseph Nadeau (Na) and is maintained at The Jackson Laboratory (J). Chromosome substitution strains or consomic strains can accelerate quantitative trait loci identification and mapping.Development
Dr. Joseph Nadeau from Case Western Reserve University developed the C57BL/6J-Chr #A/NaJ strain set by replacing individual chromosomes from the A/J donor strain into the C57BL/6J host strain using a marker-assisted series of backcrosses (Nadeau et al., 2000). The set was donated to The Jackson Laboratory in 2004.
Control Information
| Control | ||
|---|---|---|
| 000664 C57BL/6J | ||
| Considerations for Choosing Controls | ||
Related Strains
C57BL/6J-Chr#A/J/NaJ consomic panel
004388 C57BL/6J-Chr 10A/J/NaJ 004389 C57BL/6J-Chr 11A/J/NaJ 004390 C57BL/6J-Chr 12A/J/NaJ 004392 C57BL/6J-Chr 14A/J/NaJ 004393 C57BL/6J-Chr 15A/J/NaJ 004394 C57BL/6J-Chr 16A/J/NaJ 004395 C57BL/6J-Chr 17A/J/NaJ 004396 C57BL/6J-Chr 18A/J/NaJ 004397 C57BL/6J-Chr 19A/J/NaJ 004379 C57BL/6J-Chr 1A/J/NaJ 004380 C57BL/6J-Chr 2A/J/NaJ 004381 C57BL/6J-Chr 3A/J/NaJ 004382 C57BL/6J-Chr 4A/J/NaJ 004383 C57BL/6J-Chr 5A/J/NaJ 004384 C57BL/6J-Chr 6A/J/NaJ 004385 C57BL/6J-Chr 7A/J/NaJ 004386 C57BL/6J-Chr 8A/J/NaJ 004387 C57BL/6J-Chr 9A/J/NaJ 004398 C57BL/6J-Chr XA/J/NaJ 004399 C57BL/6J-Chr YA/J/NaJ 005545 C57BL/6J-mtA/J/NaJ View C57BL/6J-Chr#A/J/NaJ consomic panel (21 strains)
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 000653 BUB/BnJ 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 004385 C57BL/6J-Chr 7A/J/NaJ 000662 C57BLKS/J 000667 C57BR/cdJ 000668 C57L/J 000669 C58/J 010614 CBACa.B6-Cdh23ahl/Kjn 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
001137 129P1/ReJ 000690 129P3/J 000691 129X1/SvJ 000646 A/J 000647 A/WySnJ 003070 ALR/LtJ 003072 ALS/LtJ 002552 B6(V)-Cdh23v-2J/J 002756 B6.CAST-Cdh23Ahl+/Kjn 010615 B6.CBACa-Cdh23CBA/CaJ/Kjn 004502 B6;AKR-Lxl2/GrsrJ 002432 B6J x B6.C-H2-Kbm1/ByJ-Cdh23v-J/J 001026 BALB/cByJ 000653 BUB/BnJ 005494 C3.129S1(B6)-Grm1rcw/J 000664 C57BL/6J 004764 C57BL/6J-Cdh23v-8J/J 004819 C57BL/6J-Cdh23v-9J/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 004385 C57BL/6J-Chr 7A/J/NaJ 000662 C57BLKS/J 000667 C57BR/cdJ 000668 C57L/J 000669 C58/J 010614 CBACa.B6-Cdh23ahl/Kjn 005016 CByJ;B6-Cdh23v-10J/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 000275 V/LeJ
Additional Web Information
Genetic Quality Control Annual Report
JAX® NOTES, Spring 2004; 493. Chromosome Substitution Strain Panel: A New Tool for Quantitative Trait Loci Analysis.
Phenotype
Phenotype Information
Mouse Phenome Database
This mouse can be used to support research in many areas including:
Cdh23ahl relatedResearch Tools
Genetics Research
Gene Mapping
Gene Mapping: Tools for QTL Mapping, Segregation and Linkage Analysis
Neurobiology Research
Hearing Defects
Age related hearing loss
Sensorineural Research
Hearing Defects
Age related hearing loss
Genes & Alleles
Gene & Allele Information provided by MGI
| 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; CDHR23; 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] | ||
References
References provided by MGI
Selected Reference(s)
Nadeau JH; Singer JB; Matin A; Lander ES. 2000. Analysing complex genetic traits with chromosome substitution strains. Nat Genet 24(3):221-5. [PubMed: 10700173] [MGI Ref ID J:60756]
Additional References
Singer JB; Hill AE; Burrage LC; Olszens KR; Song J; Justice M; O'Brien WE; Conti DV; Witte JS; Lander ES; Nadeau JH. 2004. Genetic dissection of complex traits with chromosome substitution strains of mice. Science 304(5669):445-8. [PubMed: 15031436] [MGI Ref ID J:90105]
Cdh23ahl 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]
Han F; Yu H; Tian C; Chen HE; Benedict-Alderfer C; Zheng Y; Wang Q; Han X; Zheng QY. 2010. A new mouse mutant of the Cdh23 gene with early-onset hearing loss facilitates evaluation of otoprotection drugs. Pharmacogenomics J :. [PubMed: 20644563] [MGI Ref ID J:174758]
Johnson KR; Erway LC; Cook SA; Willott JF; Zheng QY. 1997. A major gene affecting age-related hearing loss in C57BL/6J mice Hear Res 114(1-2):83-92. [PubMed: 9447922] [MGI Ref ID J:44966]
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]
Johnson KR; Yu H; Ding D; Jiang H; Gagnon LH; Salvi RJ. 2010. Separate and combined effects of Sod1 and Cdh23 mutations on age-related hearing loss and cochlear pathology in C57BL/6J mice. Hear Res 268(1-2):85-92. [PubMed: 20470874] [MGI Ref ID J:163035]
Johnson KR; Zheng QY; Bykhovskaya Y; Spirina O; Fischel-Ghodsian N. 2001. A nuclear-mitochondrial DNA interaction affecting hearing impairment in mice. Nat Genet 27(2):191-4. [PubMed: 11175788] [MGI Ref ID J:67312]
Johnson KR; Zheng QY; Noben-Trauth K. 2006. Strain background effects and genetic modifiers of hearing in mice. Brain Res 1091(1):79-88. [PubMed: 16579977] [MGI Ref ID J:110459]
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]
Keithley EM; Canto C; Zheng QY; Fischel-Ghodsian N; Johnson KR. 2004. Age-related hearing loss and the ahl locus in mice. Hear Res 188(1-2):21-8. [PubMed: 14759567] [MGI Ref ID J:87783]
Liu X; Bulgakov OV; Darrow KN; Pawlyk B; Adamian M; Liberman MC; Li T. 2007. Usherin is required for maintenance of retinal photoreceptors and normal development of cochlear hair cells. Proc Natl Acad Sci U S A 104(11):4413-8. [PubMed: 17360538] [MGI Ref ID J:118927]
Manji SS; Williams LH; Miller KA; Ooms LM; Bahlo M; Mitchell CA; Dahl HH. 2011. A mutation in synaptojanin 2 causes progressive hearing loss in the ENU-mutagenised mouse strain Mozart. PLoS One 6(3):e17607. [PubMed: 21423608] [MGI Ref ID J:171701]
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; Latoche JR; Neely HR; Bennett B. 2010. Phenotype and genetics of progressive sensorineural hearing loss (Snhl1) in the LXS set of recombinant inbred strains of mice. PLoS One 5(7):e11459. [PubMed: 20628639] [MGI Ref ID J:163117]
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]
Perrin BJ; Sonnemann KJ; Ervasti JM. 2010. beta-actin and gamma-actin are each dispensable for auditory hair cell development but required for Stereocilia maintenance. PLoS Genet 6(10):e1001158. [PubMed: 20976199] [MGI Ref ID J:167543]
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]
Zilberstein Y; Liberman MC; Corfas G. 2012. Inner hair cells are not required for survival of spiral ganglion neurons in the adult cochlea. J Neurosci 32(2):405-10. [PubMed: 22238076] [MGI Ref ID J:179911]
Health & husbandry
Health & Colony Maintenance Information
Animal Health Reports
Room Number FGB27
Colony Maintenance
Mating System Sibling x Sibling (Female x Male) 01-MAR-06 Diet Information LabDiet® 5K52/5K67
Purchasing information
Pricing, Supply Level & Notes, Controls
| Pricing for USA, Canada and Mexico shipping destinations |
|
 |
Live Mice
Price (US dollars $) Gender Genotypes Provided Individual Mouse $110.00 Female or Male Homozygous
Pairs /Price (US dollars $) Pair Genotype $220.00 Homozygous x Homozygous Standard Supply
Repository-Live. The Repository Strains represent an exclusive set of over 1500 unique mouse models maintained at The Jackson Laboratory to support a vast array of research areas. The breeding colonies for Repository Strains provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. We treat orders for these strains as custom orders. Within 2 business days, we respond to each availability inquiry or order with various delivery options. Repository Strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping.
| Pricing for International shipping destinations |
|
|
Live Mice
Price (US dollars $) Gender Genotypes Provided Individual Mouse $143.00 Female or Male Homozygous
Pairs /Price (US dollars $) Pair Genotype $286.00 Homozygous x Homozygous Standard Supply
Repository-Live. The Repository Strains represent an exclusive set of over 1500 unique mouse models maintained at The Jackson Laboratory to support a vast array of research areas. The breeding colonies for Repository Strains provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. We treat orders for these strains as custom orders. Within 2 business days, we respond to each availability inquiry or order with various delivery options. Repository Strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping.
|
|
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Standard Supply
Repository-Live. The Repository Strains represent an exclusive set of over 1500 unique mouse models maintained at The Jackson Laboratory to support a vast array of research areas. The breeding colonies for Repository Strains provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. We treat orders for these strains as custom orders. Within 2 business days, we respond to each availability inquiry or order with various delivery options. Repository Strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping.
General Supply Notes
- This strain is included in the Special Mutant Stock Resource collection.
- Genomic DNA is available for this strain from the Mouse DNA Resource.
Control Information
| Control | ||
|---|---|---|
| 000664 C57BL/6J | ||
| Considerations for Choosing Controls | ||
| Control Pricing Information for Genetically Engineered Mutant Strains. | ||
Important Note
The C57BL/6J background strain is homozygous for the age related hearing loss mutation Cdh23ahl, which on this background causes progressive hearing loss with onset after 10 months of age.
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The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.Ordering Information
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