Description

Strain Information

Type Inbred Strain; Additional information on Inbred Strains. Visit our online Nomenclature tutorial. Mating System Sibling x Sibling         (Female x Male)   01-MAR-06 Breeding Considerations This strain is a challenging breeder. Species laboratory mouse H2 Haplotype d Generation F163 (17-SEP-12) Generation Definitions

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Appearance
black
Related Genotype: a/a

Important Note
This strain is homozygous for Cdh23^ahl, 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 (Lepr^db) and obese (Lep^ob) each express a much more severe phenotype on the C57BLKS/J background than on the C57BL/6J background. The Cpe^fat 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 H2^b of C57BL/6J, this strain was found homozygous for the H2^d 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   Ahr^b-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   Ahr^b-1     (11 strains)

Strains carrying   Cdh23^ahl 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   Cdh23^ahl     (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
006203	B6.129(FVB)-Ahrtm3.1Bra/J
002831	B6.129-Ahrtm1Bra/J
000130	B6.C-H17c/(HW14)ByJ
000370	B6.C-H38c/(HW119)ByJ
008599	B6.Cg-Cyp1a2/Cyp1a1tm2Dwn 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
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

Phenotypic Data

Mouse Phenome Database
Festing Inbred Strain Characteristics: C57BLKS

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

Ahr^b-1 related

Metabolism Research

Research Tools
Toxicology Research

Cdh23^ahl 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
Common Name(s)		Ah; Ahb-1; Ahb; Ahhi; Ahrb; In;
Strain of Origin		C57BL/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 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]

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]

Ahr^b-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]

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]

Cdh23^ahl 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]

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 System	Sibling 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

General Supply Notes

• Genomic DNA is available for this strain from the Mouse DNA Resource.

Important Note

This strain is homozygous for Cdh23^ahl, 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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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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