JAX Mouse Strain Datasheet - 002106

KK/HlJ

Stock No:: 002106

Inbred Strain

Repository Live

Overview

This strain serves as a model of noninsulin dependent diabetes mellitus, type 2.

Genetic overview

Genetic Background	Generation
	F107+4pF3 (21-NOV-16)

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Research Applications

Diabetes and Obesity Research
Neurobiology Research
Sensorineural Research

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Base Price

Starting at:

$224.00 Domestic price for female

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Details

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 after 10 months of age. It has been reported that the KK strain is hemolytic complement deficient (Hc⁰), however, our strains have not been tested to confirm this allele. (Cinader B, et al. 1964, J Exp Med 120:897)

Detailed Description

KK/HlJ male mice exhibit diabetic symptoms that includes hyperglycemia, hyperinsulinemia, and insulin resistance.

Development

The KK inbred strain was inbred by K Kondo in 1944. Many of the KK substrain carry the yellow (A^y ) gene. The KK/HlJ substrain, wildtype for the A^y gene, was provided by Dr. Leiselotte Herberg, Diabetes Research Institute, Dusseldorf, Germany. The current generation of inbreeding is F64+.

Selected References

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: J:89298

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Genetics

Cdh23^ahl

Allele Symbol: Cdh23^ahl

Allele Name	age related hearing loss 1
Allele Type	Spontaneous
Allele Synonym(s)	Cdh23^753A; mdfw
Gene Symbol and Name	Cdh23, cadherin 23 (otocadherin)
Gene Synonym(s)	4930542A03Rik; 4930542A03Rik; CDHR23; RIKEN cDNA 4930542A03 gene; USH1D; W; age related hearing loss 1; ahl; ahl; bob; bob; bobby; bus; bustling; mdfw; mdfw; modifier of deaf waddler; neuroscience mutagenesis facility, 112; neuroscience mutagenesis facility, 181; neuroscience mutagenesis facility, 252; nmf112; nmf112; nmf181; nmf181; nmf252; nmf252; sals; sals; salsa; v; waltzer
Strain of Origin	multiple strains
Chromosome	10
Molecular Note	Genetic complementation tests have shown allelism between the mdfw (modifier of deaf waddler) locus and the ahl locus. Further analysis has shown this is caused by a G to A transition at nucleotide position 753 of Cdh23. This hypomorphic allele causes in frame skipping of exon 7, which is predicted to delete part of the 2nd and 3rd ectodomains, and cause 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.

Disease/Phenotype

Disease Terms

Research Areas By Genotype

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

Diabetes and Obesity Research
- Hyperglycemia
  - males
- Hyperinsulinemia
  - males
- Insulin Resistance
  - males
- Type 2 Diabetes (NIDDM)
  - males
Neurobiology Research
- Hearing Defects
  - Age related hearing loss
Sensorineural Research
- Hearing Defects
  - Age related hearing loss

Genotype: Cdh23^ahl related

Neurobiology Research
- Hearing Defects
  - Age related hearing loss
Sensorineural Research
- Hearing Defects
  - Age related hearing loss

Phenotype Information

Festing Inbred Strain Characteristics: KK

References

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: J:89298

Additional References

Herberg L; Coleman DL. 1977. Laboratory animals exhibiting obesity and diabetes syndromes. Metabolism 26(1):59-99. PubMed: 834144 MGI: J:5759
Igel M; Taylor BA; Phillips SJ; Becker W; Herberg L; Joost HG. 1998. Hyperleptinemia and leptin receptor variant Asp600Asn in the obese, hyperinsulinemic KK mouse strain. J Mol Endocrinol 21(3):337-45. PubMed: 9845674 MGI: J:53637
Shafrir E. 1992. Animal models of non-insulin-dependent diabetes. Diabetes Metab Rev 8(3):179-208. PubMed: 1292911 MGI: J:4453
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: J:54812

Additional - 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: 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: 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: 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: 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: J:174758
Hurd EA; Adams ME; Layman WS; Swiderski DL; Beyer LA; Halsey KE; Benson JM; Gong TW; Dolan DF; Raphael Y; Martin DM. 2011. Mature middle and inner ears express Chd7 and exhibit distinctive pathologies in a mouse model of CHARGE syndrome. Hear Res 282(1-2):184-95. PubMed: 21875659 MGI: J:220430
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: 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: J:139223
Johnson KR; Tian C; Gagnon LH; Jiang H; Ding D; Salvi R. 2017. Effects of Cdh23 single nucleotide substitutions on age-related hearing loss in C57BL/6 and 129S1/Sv mice and comparisons with congenic strains. Sci Rep 7:44450. PubMed: 28287619 MGI: J:241137
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: 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: 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: 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: 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: 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: 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: 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: 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: 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: J:88012
Nagtegaal AP; Rainey RN; van der Pluijm I; Brandt RM; van der Horst GT; Borst JG; Segil N. 2015. Cockayne syndrome group B (csb) and group a (csa) deficiencies predispose to hearing loss and cochlear hair cell degeneration in mice. J Neurosci 35(10):4280-6. PubMed: 25762674 MGI: J:219993
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: 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: 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: 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: 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: 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: J:117746
Watson CJ; Tempel BL. 2013. A new Atp2b2 deafwaddler allele, dfw(i5), interacts strongly with Cdh23 and other auditory modifiers. Hear Res 304:41-8. PubMed: 23792079 MGI: J:220660
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: 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: 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: J:179911

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The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks.

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Genetic overview

Research Applications

Base Price

Important Note

Detailed Description

Development

Selected References

Cdh23ahl

Allele Symbol: Cdh23ahl

Disease Terms

Research Areas By Genotype

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

Genotype: Cdh23ahl related

Phenotype Information

References

Additional References

Additional - Cdh23ahl related

Genotyping Protocols

Dietary Information

Mating System

Appearance

Animal Health Reports

Live Mouse

Cryorecovery - Pricing

Progeny testing is not required

RELATED PRODUCTS AND SERVICES

Payment Terms and Conditions

The Jackson Laboratory's Genotype Promise

Terms of Use

Licensing Information

JAX® Mice, Products & Services Conditions of Use

No Warranty

Credit for PRODUCTS or SERVICES

Animal Care and Use for SERVICES

No Liability

Cdh23^ahl

Allele Symbol: Cdh23^ahl

Genotype: Cdh23^ahl related

Additional - Cdh23^ahl related