Description

Strain Information

Type Inbred Strain; Additional information on Inbred Strains. Mating System Sibling x Sibling         (Female x Male) Species laboratory mouse H2 Haplotype d Generation F147 (03-JAN-08)

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   Cdh23^ahl allele

001137	129P1/ReJ
000690	129P3/J
002065	129T2/SvEmsJ
000691	129X1/SvJ
000646	A/J
000647	A/WySnJ
003070	ALR/LtJ
003072	ALS/LtJ
004502	B6;AKR-Lxl2/J
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/J
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
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 Cdh23

008288	B6(Cg)-Cdh23v-11J/J
002756	B6.CAST-Cdh23Ahl+/Kjn
002432	B6J x B6.C-H2bm1/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     (8 strains)

Additional Web Information

Genetic Quality Control Annual Report
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 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
Vestibular and Hearing Defects (Age related hearing loss)

Research Tools
General Purpose

Sensorineural Research
Vestibular and Hearing Defects (Age related hearing loss)

Cdh23^ahl related

Neurobiology Research
Vestibular and Hearing Defects (Age related hearing loss)

Sensorineural Research
Vestibular and Hearing Defects (Age related hearing loss)

Genes & Alleles

Gene & Allele Information

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; 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/LyJ, 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

This strain will not have a genotyping protocol or one is not currently available.

Helpful Links

Optimizing PCR Protocols

References

References

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]

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]

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]

Cdh23^ahl related

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]

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; 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]

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]

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]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           AX4
Room Number           AX8

Colony Maintenance

Mating System	Sibling x Sibling         (Female x Male)
Diet Information	LabDiet® 5K52/5K67

Purchasing information

Pricing, Supply Level & Notes, Controls, General Terms & Conditions

Pricing

Supply Details

Standard Supply	Level 3. Up to 50 mice. Larger quantities or custom orders arranged upon request.
Supply Notes	Shipped at a specific age in weeks. Mice at a precise age in days, littermates and retired breeders are also available. Strains that must be genotyped are not available until five to seven weeks of age.
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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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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