Strain Name:

C57BLKS/J

Stock Number:

000662

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Availability:

Level 4

Common Names: BKS;     C57BL/Ks;     C57Kaliss;     Black Kaliss J;    
C57BLKS/J is closely related to C57BL/6J, but the strains are phenotypically distinct. Differences include more severe diet-induced atherosclerotic lesions in C57BLKS/J mice, low total cholesterol and low HDL cholesterol when fed a normal diet, and high total cholesterol and HDL cholesterol in an atherogenic diet. C57BLKS/J mice have a relatively high percentage of reticulocytes per total number of erythrocytes, high white blood cell count per volume, with a high percentage of lymphocytes and basophils and a low percentage of neutrophils per total number of leukocytes. C57BLKS/J mice exhibit age related hearing loss.

Description

Strain Information

Type Inbred Strain;
Additional information on Inbred Strains.
Visit our online Nomenclature tutorial.
Mating SystemSibling x Sibling         (Female x Male)   01-MAR-06
Breeding Considerations This strain is a challenging breeder.
Specieslaboratory mouse
H2 Haplotyped
GenerationF167 (05-AUG-14)
Generation Definitions

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Appearance
black
Related Genotype: a/a

Important Note
This strain is homozygous for Cdh23ahl, the age related hearing loss 1 mutation, which on this background results in progressive hearing loss with onset prior to three months of age.

Description
Although C57BLKS/J is estimated to have more than 70% of its genome derived from C57BL/6J, these strains are phenotypically distinct. Diet-induced atherosclerotic lesions are much more severe in C57BLKS/J than in C57BL/6J or many other inbred strains. Compared with a panel of inbred strains, C57BLKS/J was found to have low total cholesterol and low HDL cholesterol when fed a normal diet and high total cholesterol and HDL cholesterol in an atherogenic diet. Paigen et al. found high levels of plasma bile salts in C57BLKS/J females after eight weeks on an atherogenic diet. The mutations diabetes (Leprdb) and obese (Lepob) each express a much more severe phenotype on the C57BLKS/J background than on the C57BL/6J background. The Cpefat mutation causes severe obesity, hyperinsulinemia, and hyperglycemia on the C57BLKS/J background rather than the hyperinsulinemia, and mild obesity without hyperglycemia found on the HRS/J background. In a comparative study of 43 inbred strains, Barker and Peters found that C57BLKS/J had a relatively high percentage of reticulocytes per total number of erythrocytes, high white blood cell count per volume, with a high percentage of lymphocytes and basophils and a low percentage of neutrophils per total number of leukocytes. C57BLKS/J mice exhibit age related hearing loss by three months of age.

Development
In October, 1947, while at The Sloan-kettering Institute, Dr. N. Kaliss obtained a C57BL/6J male from The Jackson Laboratory and obtained a female, which was reported to be one generation removed from the male, from a pen-bred colony of C57BL/6J of Dr. J. J. Biesele. Dr. Kaliss bred these together and maintained an inbreeding line of what he thought were C57BL/6J and brought them back with him to The Jackson Laboratory in 1948. He found that with continued inbreeding this strain rejected the C57BL/6-derived sarcoma E0771. It was subsequently determined that this strain had a genetic contamination untraced in origin. Rather than the H2b of C57BL/6J, this strain was found homozygous for the H2d haplotype found also in DBA/2J. Polymorphisms have been assessed to characterize this contamination and Mao et al., following on work of Naggert et al. and Slingsby et al., reported finding that the C57BLKS/J genome derived from 71% C57BL/6J, 25% DBA/2J, and 4% from a combination of C57BL/10J, a 129 source, and possibly some other undefined source. Clusters of 129-like alleles were found on Chr 4 and 15, C57BL/10-like alleles were found on Chr 11 and elsewhere in the genome, and additional unique alleles were also identified.

Related Strains

Strains carrying   Ahrb-1 allele
000136   B6.C-H34c/(HW22)ByJ
000663   C57BL/6By
001139   C57BL/6ByJ
000664   C57BL/6J
000667   C57BR/cdJ
000668   C57L/J
000669   C58/J
000351   CXB1/ByJ
000356   CXB6/ByJ
002937   D2.B6-Ahrb-1/J
000677   MA/MyJ
View Strains carrying   Ahrb-1     (11 strains)

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
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
004391   C57BL/6J-Chr 13A/J/NaJ
004385   C57BL/6J-Chr 7A/J/NaJ
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
View Strains carrying   Cdh23ahl     (41 strains)

Strains carrying other alleles of Ahr
000690   129P3/J
000645   A/HeJ
000646   A/J
000648   AKR/J
002920   B6(D2N).Spretus-Ahrb-3/J
002831   B6.129-Ahrtm1Bra/J
000130   B6.C-H17c/(HW14)ByJ
000370   B6.C-H38c/(HW119)ByJ
008599   B6.Cg-Del(9Cyp1a2-Cyp1a1)1Dwn Ahrd Tg(CYP1A1,CYP1A2)1Dwn/DwnJ
002921   B6.D2N-Ahrd/J
002727   B6;129-Ahrtm1Bra/J
001026   BALB/cByJ
000652   BDP/J
000653   BUB/BnJ
000659   C3H/HeJ
000926   CAROLI/EiJ
000928   CAST/EiJ
000656   CBA/J
000657   CE/J
000352   CXB2/ByJ
000353   CXB3/ByJ
000354   CXB4/ByJ
000355   CXB5/ByJ
000357   CXB7/ByJ
000671   DBA/2J
000673   HRS/J
000674   I/LnJ
000675   LG/J
000676   LP/J
000550   MOLF/EiJ
000684   NZB/BlNJ
000679   P/J
000930   PERA/EiJ
000726   RBF/DnJ
000682   RF/J
000644   SEA/GnJ
000280   SF/CamEiJ
000686   SJL/J
001146   SPRET/EiJ
000688   ST/bJ
006203   STOCK Ahrtm3.1Bra/J
000689   SWR/J
000693   WC/ReJ KitlSl/J
000933   YBR/EiJ
View Strains carrying other alleles of Ahr     (44 strains)

Strains carrying other alleles of Cdh23
002552   B6(V)-Cdh23v-2J/J
002756   B6.CAST-Cdh23Ahl+/Kjn
010615   B6.CBACa-Cdh23CBA/CaJ/Kjn
002432   B6J x B6.C-H2-Kbm1/ByJ-Cdh23v-J/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     (8 strains)

Additional Web Information

JAX® NOTES, April 1988; 433. H-2 Haplotypes of Mice from Jackson Laboratory Production Colonies.
JAX® NOTES, Fall 1995; 463. Inbred C57 Black Mice: Microphthalmia and Ocular Infections.
JAX® NOTES, Spring 2003; 489. Malocclusion in the Laboratory Mouse.
JAX® NOTES, Summer 2003; 490. Hydrocephalus in Laboratory Mice.

Phenotype

Phenotype Information

View Phenotypic Data

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Deafness, Autosomal Recessive 12; DFNB12   (CDH23)
Usher Syndrome, Type ID; USH1D   (CDH23)
View Research Applications

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

Diabetes and Obesity Research
Type 2 Diabetes (NIDDM)
      diabetes susceptible background strain

Neurobiology Research
Hearing Defects
      Age related hearing loss

Research Tools
General Purpose

Sensorineural Research
Hearing Defects
      Age related hearing loss

Ahrb-1 related

Metabolism Research

Research Tools
Toxicology Research

Cdh23ahl related

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 Ahrb-1
Allele Name b-1 variant
Allele Type Not Applicable (Not Applicable)
Common Name(s) Ah; Ahb-1; Ahb; Ahhi; Ahrb; In;
Strain of OriginC57BL/6J
Gene Symbol and Name Ahr, aryl-hydrocarbon receptor
Chromosome 12
Gene Common Name(s) Ah; Ahh; Ahre; In; aromatic hydrocarbon responsiveness; aryl hydrocarbon hydroxylase; bHLHe76; dioxin receptor; inflammatory reactivity;
General Note C57BL/6 carries the responsive Ahrb allele; DBA/2 carries nonresponsive Ahrd. Heterozygotes (Ahrb/Ahrd) are responsive (J:5282). Later work identified a second (J:8895) and later a third (J:22144) allele conferring response. Thus the allele in C57, C58, and MA/My strains is now Ahrb-1; Ahrb-2 is carried by BALB/cBy, A, and C3H; and Ahrb-3 by Mus spretus, M. caroli, and MOLF/Ei. The nonresponsive strains AKR, DBA/2, and 129 carry Ahrd (J:22144). Nucleotide and amino acid sequence differences between Ahrb-1 and Ahrd have been determined (J:17460).

Strain of origin - this allele was found in C57BL/6, C58/J, C57BR, MA/My strains

Molecular Note This allele encodes a high affinity, relatively heat stabile, 95 kDa receptor. PCR sequencing of cDNA revealed ten nucleotide differences between the coding sequences of the DBA/2J and C57BL/6J receptors. Five of the ten differences would cause amino acid changes. One of these, a C to T transition in exon 11 would change the arginine codon in the DBA/2J allele to an opal termination codon in the C57BL/6J allele. This change would prevent the 43 amino acid extension of mRNA translation predicted for the DBA/2J allele and account for the smaller size of the peptide produced by this allele (95 kDa vs 104 kDa for the DBA/2J allele). A second C to T transition changes a proline codon in the DBA/2J allele to leucine codon in the C57BL/6J allele, and would likely change secondary structure of the peptide and thus ligand affinity. [MGI Ref ID J:15153] [MGI Ref ID J:17460] [MGI Ref ID J:477]
 
Allele Symbol Cdh23ahl
Allele Name age related hearing loss 1
Allele Type QTL
Common Name(s) Cdh23753A; mdfw;
Strain of Originmultiple 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]

Genotyping

Genotyping Information

Inbred mouse strains are maintained through sibling (sister x brother) matings; no genotyping required.

Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Collin GB; Maddatu TP; Sen S; Naggert JK. 2005. Genetic modifiers interact with Cpefat to affect body weight, adiposity, and hyperglycemia. Physiol Genomics 22(2):182-90. [PubMed: 15870393]  [MGI Ref ID J:100832]

Leiter EH; Chapman HD; Coleman DL. 1989. The influence of genetic background on the expression of mutations at the diabetes locus in the mouse. V. Interaction between the db gene and hepatic sex steroid sulfotransferases correlates with gender-dependent susceptibility to hyperglycemia. Endocrinology 124(2):912-22. [PubMed: 2912706]  [MGI Ref ID J:26013]

Mao HZ; Roussos ET; Peterfy M. 2006. Genetic analysis of the diabetes-prone C57BLKS/J mouse strain reveals genetic contribution from multiple strains. Biochim Biophys Acta 1762(4):440-6. [PubMed: 16481151]  [MGI Ref ID J:108762]

Naggert JK; Mu JL; Frankel W; Bailey DW; Paigen B. 1995. Genomic analysis of the C57BL/Ks mouse strain. Mamm Genome 6(2):131-3. [PubMed: 7766997]  [MGI Ref ID J:22800]

Petkov PM; Cassell MA; Sargent EE; Donnelly CJ; Robinson P; Crew V; Asquith S; Haar RV; Wiles MV. 2004. Development of a SNP genotyping panel for genetic monitoring of the laboratory mouse. Genomics 83(5):902-11. [PubMed: 15081119]  [MGI Ref ID J:89298]

Surwit RS; Seldin MF; Kuhn CM; Secor C; Feinglos MN. 1994. Diet-induced obesity and diabetes in C57BL/6J and C57BL/KsJ mice Mouse Genome 92:523-8.  [MGI Ref ID J:40713]

Zheng QY; Johnson KR; Erway LC. 1999. Assessment of hearing in 80 inbred strains of mice by ABR threshold analyses. Hear Res 130(1-2):94-107. [PubMed: 10320101]  [MGI Ref ID J:54812]

Additional References

Coleman DL; Hummel KP. 1973. The influence of genetic background on the expression of the obese (Ob) gene in the mouse. Diabetologia 9(4):287-93. [PubMed: 4588246]  [MGI Ref ID J:5400]

Goren HJ; Kulkarni RN; Kahn CR. 2004. Glucose homeostasis and tissue transcript content of insulin signaling intermediates in four inbred strains of mice: C57BL/6, C57BLKS/6, DBA/2, and 129X1. Endocrinology 145(7):3307-23. [PubMed: 15044376]  [MGI Ref ID J:90745]

Graff RJ. 1970. Polymorphism of histocompatibility genes in the mouse. Transplant Proc 2(1):15-23. [PubMed: 4107291]  [MGI Ref ID J:5237]

Peters LL; Lambert AJ; Zhang W; Churchill GA; Brugnara C; Platt OS. 2006. Quantitative trait loci for baseline erythroid traits. Mamm Genome 17(4):298-309. [PubMed: 16596451]  [MGI Ref ID J:107220]

Peters LL; Zhang W; Lambert AJ; Brugnara C; Churchill GA; Platt OS. 2005. Quantitative trait loci for baseline white blood cell count, platelet count, and mean platelet volume. Mamm Genome 16(10):749-63. [PubMed: 16261417]  [MGI Ref ID J:102636]

Poland A; Glover E. 1990. Characterization and strain distribution pattern of the murine Ah receptor specified by the Ahd and Ahb-3 alleles. Mol Pharmacol 38(3):306-12. [PubMed: 2169579]  [MGI Ref ID J:34840]

Wejman JC; Taylor BA; Jenkins NA; Copeland NG. 1984. Endogenous xenotropic murine leukemia virus-related sequences map to chromosomal regions encoding mouse lymphocyte antigens. J Virol 50(1):237-47. [PubMed: 6321791]  [MGI Ref ID J:7348]

Ahrb-1 related

Benedict WF; Considine N; Nebert DW. 1973. Genetic differences in aryl hydrocarbon hydroxylase induction and benzo(a)pyrene-produced tumorigenesis in the mouse. Mol Pharmacol 9(2):266-77. [PubMed: 4123113]  [MGI Ref ID J:84312]

Boobis AR; Nebert DW. 1976. Genetic differences in the metabolism of carcinogens and in the binding of benzo (a) pyrene metabolites to DNA. Adv Enzyme Regul 15:339-62. [PubMed: 1030186]  [MGI Ref ID J:12156]

Bradfield CA; Glover E; Poland A. 1991. Purification and N-terminal amino acid sequence of the Ah receptor from the C57BL/6J mouse. Mol Pharmacol 39(1):13-9. [PubMed: 1846217]  [MGI Ref ID J:84440]

Burbach KM; Poland A; Bradfield CA. 1992. Cloning of the Ah-receptor cDNA reveals a distinctive ligand-activated transcription factor. Proc Natl Acad Sci U S A 89(17):8185-9. [PubMed: 1325649]  [MGI Ref ID J:2256]

Castro DJ; Lohr CV; Fischer KA; Pereira CB; Williams DE. 2008. Lymphoma and lung cancer in offspring born to pregnant mice dosed with dibenzo[a,l]pyrene: the importance of in utero vs. lactational exposure. Toxicol Appl Pharmacol 233(3):454-8. [PubMed: 18848954]  [MGI Ref ID J:143604]

Chang C; Smith DR; Prasad VS; Sidman CL; Nebert DW; Puga A. 1993. Ten nucleotide differences, five of which cause amino acid changes, are associated with the Ah receptor locus polymorphism of C57BL/6 and DBA/2 mice. Pharmacogenetics 3(6):312-21. [PubMed: 8148872]  [MGI Ref ID J:17460]

Curran CP; Miller KA; Dalton TP; Vorhees CV; Miller ML; Shertzer HG; Nebert DW. 2006. Genetic differences in lethality of newborn mice treated in utero with coplanar versus non-coplanar hexabromobiphenyl. Toxicol Sci 89(2):454-64. [PubMed: 16291824]  [MGI Ref ID J:113285]

Ema M; Sogawa K; Watanabe N; Chujoh Y; Matsushita N; Gotoh O; Funae Y; Fujii-Kuriyama Y. 1992. cDNA cloning and structure of mouse putative Ah receptor. Biochem Biophys Res Commun 184(1):246-53. [PubMed: 1314586]  [MGI Ref ID J:477]

Gielen JE; Goujon FM; Nebert DW. 1972. Genetic regulation of aryl hydrocarbon hydroxylase induction. II. Simple Mendelian expression in mouse tissues in vivo. J Biol Chem 247(4):1125-37. [PubMed: 4110756]  [MGI Ref ID J:84250]

Goujon FM; Nebert DW; Gielen JE. 1972. Genetic expression of aryl hydrocarbon hydroxylase induction. IV. Interaction of various compounds with different forms of cytochrome P-450 and the effect on benzo(a)pyrene metabolism in vitro. Mol Pharmacol 8(6):667-80. [PubMed: 4118365]  [MGI Ref ID J:84252]

Hansen DA; Esakky P; Drury A; Lamb L; Moley KH. 2014. The aryl hydrocarbon receptor is important for proper seminiferous tubule architecture and sperm development in mice. Biol Reprod 90(1):8. [PubMed: 24174576]  [MGI Ref ID J:210360]

Harper PA; Golas CL; Okey AB. 1991. Ah receptor in mice genetically nonresponsive for cytochrome P4501A1 induction: cytosolic Ah receptor, transformation to the nuclear binding state, and induction of aryl hydrocarbon hydroxylase by halogenated and nonhalogenated aromatic hydrocarbons in embryonic tissues and cells. Mol Pharmacol 40(5):818-26. [PubMed: 1658612]  [MGI Ref ID J:2134]

Kerley-Hamilton JS; Trask HW; Ridley CJ; Dufour E; Lesseur C; Ringelberg CS; Moodie KL; Shipman SL; Korc M; Gui J; Shworak NW; Tomlinson CR. 2012. Inherent and benzo[a]pyrene-induced differential aryl hydrocarbon receptor signaling greatly affects life span, atherosclerosis, cardiac gene expression, and body and heart growth in mice. Toxicol Sci 126(2):391-404. [PubMed: 22228805]  [MGI Ref ID J:183715]

Kouri RE; Rude TH; Joglekar R; Dansette PM; Jerina DM; Atlas SA; Owens IS; Nebert DW. 1978. 2,3,7,8-tetrachlorodibenzo-p-dioxin as cocarcinogen causing 3-methylcholanthrene-initiated subcutaneous tumors in mice genetically 'nonresponsive' at Ah locus. Cancer Res 38(9):2777-83. [PubMed: 679184]  [MGI Ref ID J:84318]

Levova K; Moserova M; Nebert DW; Phillips DH; Frei E; Schmeiser HH; Arlt VM; Stiborova M. 2012. NAD(P)H:quinone oxidoreductase expression in Cyp1a-knockout and CYP1A-humanized mouse lines and its effect on bioactivation of the carcinogen aristolochic acid I. Toxicol Appl Pharmacol 265(3):360-7. [PubMed: 22982977]  [MGI Ref ID J:192865]

Lew BJ; Manickam R; Lawrence BP. 2011. Activation of the aryl hydrocarbon receptor during pregnancy in the mouse alters mammary development through direct effects on stromal and epithelial tissues. Biol Reprod 84(6):1094-102. [PubMed: 21270426]  [MGI Ref ID J:173706]

Moriguchi T; Motohashi H; Hosoya T; Nakajima O; Takahashi S; Ohsako S; Aoki Y; Nishimura N; Tohyama C; Fujii-Kuriyama Y; Yamamoto M. 2003. Distinct response to dioxin in an arylhydrocarbon receptor (AHR)-humanized mouse. Proc Natl Acad Sci U S A 100(10):5652-7. [PubMed: 12730383]  [MGI Ref ID J:132380]

Nebert DW; Atlas SA; Guenthner TM; Kouri RE. 1978. The Ah locus: genetic regulation of the enzymes which metabolize polycyclic hydrocarbons and the risk of cancer. In: Polycyclic Hydrocarbons and Cancer: Chemistry, Molecular Biology and Environment. Academic Press, New York.  [MGI Ref ID J:30693]

Nebert DW; Considine N; Owens IS. 1973. Genetic expression of aryl hydrocarbon hydroxylase induction. VI. Control of other aromatic hydrocarbon-inducible mono-oxygenase activities at or near the same genetic locus. Arch Biochem Biophys 157(1):148-59. [PubMed: 4716952]  [MGI Ref ID J:84313]

Nebert DW; Gelboin HV. 1969. The in vivo and in vitro induction of aryl hydrocarbon hydroxylase in mammalian cells of different species, tissues, strains, and developmental and hormonal states. Arch Biochem Biophys 134(1):76-89. [PubMed: 4981257]  [MGI Ref ID J:84248]

Nebert DW; Gielen JE. 1972. Genetic regulation of aryl hydrocarbon hydroxylase induction in the mouse. Fed Proc 31(4):1315-25. [PubMed: 4114109]  [MGI Ref ID J:5282]

Nebert DW; Gielen JE; Goujon FM. 1972. Genetic expression of aryl hydrocarbon hydroxylase induction. 3. Changes in the binding of n-octylamine to cytochrome P-450. Mol Pharmacol 8(6):651-66. [PubMed: 4118364]  [MGI Ref ID J:84251]

Nebert DW; Goujon FM; Gielen JE. 1972. Aryl hydrocarbon hydroxylase induction by polycyclic hydrocarbons: simple autosomal dominant trait in the mouse. Nat New Biol 236(65):107-10. [PubMed: 4502804]  [MGI Ref ID J:84249]

Nebert DW; Robinson JR; Niwa A; Kumaki K; Poland AP. 1975. Genetic expression of aryl hydrocarbon hydroxylase activity in the mouse. J Cell Physiol 85(2 Pt 2 Suppl 1):393-414. [PubMed: 1091656]  [MGI Ref ID J:84317]

Niwa A; Kumaki K; Nebert DW; Poland AP. 1975. Genetic expression of aryl hydrocarbon hydroxylase activity in the mouse. Distinction between the 'responsive' homozygote and heterozygote at the Ah locus. Arch Biochem Biophys 166(2):559-64. [PubMed: 1119809]  [MGI Ref ID J:84316]

Nukaya M; Lin BC; Glover E; Moran SM; Kennedy GD; Bradfield CA. 2010. The aryl hydrocarbon receptor-interacting protein (AIP) is required for dioxin-induced hepatotoxicity but not for the induction of the Cyp1a1 and Cyp1a2 genes. J Biol Chem 285(46):35599-605. [PubMed: 20829355]  [MGI Ref ID J:166864]

Okey AB; Vella LM; Harper PA. 1989. Detection and characterization of a low affinity form of cytosolic Ah receptor in livers of mice nonresponsive to induction of cytochrome P1-450 by 3-methylcholanthrene. Mol Pharmacol 35(6):823-30. [PubMed: 2543914]  [MGI Ref ID J:27899]

Poel WE; Stanton D; Peters E; Wade HO. 1958. Comparative susceptibilities of seven inbred strains of mice to qualified applications of 3, 4-benzpyrene Proc Am Assoc Cancer Res 2:335.  [MGI Ref ID J:84245]

Poland A; Bradfield C. 1992. A brief review of the Ah locus. Tohoku J Exp Med 168(2):83-7. [PubMed: 1339107]  [MGI Ref ID J:12546]

Poland A; Glover E. 1990. Characterization and strain distribution pattern of the murine Ah receptor specified by the Ahd and Ahb-3 alleles. Mol Pharmacol 38(3):306-12. [PubMed: 2169579]  [MGI Ref ID J:34840]

Poland A; Glover E; Kende AS. 1976. Stereospecific, high affinity binding of 2,3,7,8-tetrachlorodibenzo-p-dioxin by hepatic cytosol. Evidence that the binding species is receptor for induction of aryl hydrocarbon hydroxylase. J Biol Chem 251(16):4936-46. [PubMed: 956169]  [MGI Ref ID J:84247]

Poland A; Glover E; Taylor BA. 1987. The murine Ah locus: a new allele and mapping to chromosome 12. Mol Pharmacol 32(4):471-8. [PubMed: 2823093]  [MGI Ref ID J:8895]

Poland A; Palen D; Glover E. 1994. Analysis of the four alleles of the murine aryl hydrocarbon receptor. Mol Pharmacol 46(5):915-21. [PubMed: 7969080]  [MGI Ref ID J:22144]

Poland AP; Glover E; Robinson JR; Nebert DW. 1974. Genetic expression of aryl hydrocarbon hydroxylase activity. Induction of monooxygenase activities and cytochrome P1-450 formation by 2,3,7,8-tetrachlorodibenzo-p-dioxin in mice genetically 'nonresponsive' to other aromatic hydrocarbons. J Biol Chem 249(17):5599-606. [PubMed: 4370044]  [MGI Ref ID J:84314]

Robinson JR; Considine N; Nebert DW. 1974. Genetic expression of aryl hydrocarbon hydroxylase induction. Evidence for the involvement of other genetic loci. J Biol Chem 249(18):5851-9. [PubMed: 4413562]  [MGI Ref ID J:84315]

Schmid FA; Demetriades MS; Schabel FM 3rd; Tarnowski GS. 1967. Toxicity of several cancerigenic polycyclic hydrocarbons and other agents in AKR and C57BL-6 mice. Cancer Res 27(3):563-7. [PubMed: 6021514]  [MGI Ref ID J:84246]

Schmid FA; Elmer I; Tarnowski GS. 1969. Genetic determination of differential inflammatory reactivity and subcutaneous tumor susceptibility of AKR-J and C57BL-6J mice to 7,12-dimethylbenz- [a]anthracene. Cancer Res 29(8):1585-9. [PubMed: 5807232]  [MGI Ref ID J:34134]

Schmid FA; Pena RC; Robinson W; Tarnowski GS. 1967. Toxicity of intraperitoneal injections of 7, 12-dimethylbenz[a]anthracene in inbred mice. Cancer Res 27(3):558-62. [PubMed: 6021513]  [MGI Ref ID J:26440]

Schmidt JV; Carver LA; Bradfield CA. 1993. Molecular characterization of the murine Ahr gene. Organization, promoter analysis, and chromosomal assignment. J Biol Chem 268(29):22203-9. [PubMed: 8408082]  [MGI Ref ID J:15153]

Smith AG; Clothier B; Robinson S; Scullion MJ; Carthew P; Edwards R; Luo J; Lim CK; Toledano M. 1998. Interaction between iron metabolism and 2,3,7,8-tetrachlorodibenzo-p-dioxin in mice with variants of the Ahr gene: a hepatic oxidative mechanism. Mol Pharmacol 53(1):52-61. [PubMed: 9443932]  [MGI Ref ID J:45850]

Stiborova M; Levova K; Barta F; Shi Z; Frei E; Schmeiser HH; Nebert DW; Phillips DH; Arlt VM. 2012. Bioactivation versus detoxication of the urothelial carcinogen aristolochic acid I by human cytochrome P450 1A1 and 1A2. Toxicol Sci 125(2):345-58. [PubMed: 22086975]  [MGI Ref ID J:183662]

Taylor BA. 1971. Strain distribution and linkage tests of 7,12-dimethylbenzanthracene (DMBA) inflammatory response in mice. Life Sci I 10(19):1127-34. [PubMed: 5132702]  [MGI Ref ID J:5244]

Thomas PE; Hutton JJ; Taylor BA. 1973. Genetic relationship between aryl hydrocarbon hydroxylase inducibility and chemical carcinogen induced skin ulceration in mice. Genetics 74(4):655-9. [PubMed: 4750810]  [MGI Ref ID J:5387]

Thomas PE; Kouri RE; Hutton JJ. 1972. The genetics of aryl hydrocarbon hydroxylase induction in mice: a single gene difference between C57BL-6J and DBA-2J. Biochem Genet 6(2):157-68. [PubMed: 4666754]  [MGI Ref ID J:31977]

Yu Z; Mahadevan B; Lohr CV; Fischer KA; Louderback MA; Krueger SK; Pereira CB; Albershardt DJ; Baird WM; Bailey GS; Williams DE. 2006. Indole-3-carbinol in the maternal diet provides chemoprotection for the fetus against transplacental carcinogenesis by the polycyclic aromatic hydrocarbon dibenzo[a,l]pyrene. Carcinogenesis 27(10):2116-23. [PubMed: 16704990]  [MGI Ref ID J:113356]

Cdh23ahl related

Bosco A; Crish SD; Steele MR; Romero CO; Inman DM; Horner PJ; Calkins DJ; Vetter ML. 2012. Early reduction of microglia activation by irradiation in a model of chronic glaucoma. PLoS One 7(8):e43602. [PubMed: 22952717]  [MGI Ref ID J:191663]

Davis 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]

Fetoni AR; Picciotti PM; Paludetti G; Troiani D. 2011. Pathogenesis of presbycusis in animal models: a review. Exp Gerontol 46(6):413-25. [PubMed: 21211561]  [MGI Ref ID J:186964]

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]

Kane KL; Longo-Guess CM; Gagnon LH; Ding D; Salvi RJ; Johnson KR. 2012. Genetic background effects on age-related hearing loss associated with Cdh23 variants in mice. Hear Res 283(1-2):80-8. [PubMed: 22138310]  [MGI Ref ID J:183757]

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]

Perrin BJ; Strandjord DM; Narayanan P; Henderson DM; Johnson KR; Ervasti JM. 2013. beta-Actin and Fascin-2 Cooperate to Maintain Stereocilia Length. J Neurosci 33(19):8114-21. [PubMed: 23658152]  [MGI Ref ID J:197137]

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]

Zheng QY; Scarborough JD; Zheng Y; Yu H; Choi D; Gillespie PG. 2012. Digenic inheritance of deafness caused by 8J allele of myosin-VIIA and mutations in other Usher I genes. Hum Mol Genet 21(11):2588-98. [PubMed: 22381527]  [MGI Ref ID J:183898]

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           AX4

Colony Maintenance

Mating SystemSibling x Sibling         (Female x Male)   01-MAR-06
Breeding Considerations This strain is a challenging breeder.
Diet Information LabDiet® 5K52/5K67

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


Pricing for USA, Canada and Mexico shipping destinations View International Pricing
View pricing for U.S.A., Canada and Mexico shipping destinations

Standard Supply

Level 4. Up to 10 mice. Larger quantities or custom orders arranged upon request. Expected delivery up to one to three months.

Supply Notes

  • Shipped at a specific age in weeks. Mice at a precise age in days, littermates and retired breeders are also available.
Pricing for International shipping destinations View USA Canada and Mexico Pricing
View pricing for International shipping destinations

Standard Supply

Level 4. Up to 10 mice. Larger quantities or custom orders arranged upon request. Expected delivery up to one to three months.

Supply Notes

  • Shipped at a specific age in weeks. Mice at a precise age in days, littermates and retired breeders are also available.
View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Level 4. Up to 10 mice. Larger quantities or custom orders arranged upon request. Expected delivery up to one to three months.

Important Note

This strain is homozygous for Cdh23ahl, the age related hearing loss 1 mutation, which on this background results in progressive hearing loss with onset prior to three months of age.

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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.


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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.
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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.

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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.

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