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Strain Name:

WBB6F1/J-KitW/KitW-v/J

Stock Number:

100410

Availability:

Level 2


General Terms and Conditions

Former Name      WBB6F1/J-KitW/KitW-v    (Changed: 23-FEB-06 )
Genes & Alleles   Kit;   KitW-v;   KitW;   Pde6b;   Pde6brd1;


Product Information

Strain Details

Type *F1 Hybrid
Additional information on Hybrid Strains.
Type JAX® GEMM® Strain - Mutant Stock
Additional information on JAX® GEMM® Strains.
Type JAX® GEMM® Strain - Spontaneous Mutation
Specieslaboratory mouse

Appearance
black eyed, white coat, affected
Related Genotype: a/a KitW/KitW-v

black with white belly, spot occasionally has white head blaze, tail has white tip, affected
Related Genotype: a/a KitW/+

grey with light belly and white spot, light tail, affected
Related Genotype: a/a KitW-v/+

black, unaffected
Related Genotype: a/a +/+

Important Note
This strain is heterozygous for the retinal degeneration allele Pde6brd1.

Strain Description
Kit mutant mice possess pleiotropic defects in pigment-forming cells, germ cells, RBC's and mast cells. In addition, they exhibit impaired resistance to parasitic infection and an intrinsic progenitor cell defect. KitW-v homozygotes resemble KitW homozygotes in color, anemia, and germ cells, but many of them survive to maturity. The lack of germ cells in mutant mice leads to the development of some ovarian tumors (mesotheliomas and granulosa cell), associated with an overproduction of pituitary gonadotropic hormone. KitW/KitW-v double heterozygotes are viable but sterile because of germ cell deficiency. They are also mast cell deficient. KitW/KitW-v double heterozygotes lack intermediate cells, derived from melanoblasts, in the stria vascularis resulting in endocochlear degeneration, loss of endocochlear potential, and hearing impairment.

Mammalian Phenotype Terms assigned by genotype

KitW/KitW-v

        involves: C57BL/6 * WB
  • digestive/alimentary phenotype
  • peptic ulcer (J:6393)
    • 40% developed prepyloric ulcer, however no duodenal ulcers were found
  • tumorigenesis
  • papilloma (J:6393)
    • 40% developed forestomach papillomas
    • esophageal papilloma (J:6393)
      • observed papillomas in the lower end of the esophagus but not in the upper two-thirds of the esophagus
  • immune system phenotype
  • abnormal response to transplant (J:6084)
    • after receiving splenocytes from Kitl/Kitl donors, 15 weeks later, mice exhibit significant increase in mast cell numbers in skin, stomach and mesentery
    • after receiving skin grafts from Kitl/Kitl donors, no increase in mast cell number in skin is seen, compared to increase observed in reciprocal transplant

KitW/KitW-v

        (WB/ReJ KitW x C57BL/6J-KitW-v/J)F1-KitW/KitW-v/J
  • life span-post-weaning/aging
  • abnormal induced morbidity/mortality (J:113500)
    • significantly higher mortality is exhibited by mutants (88%) by day 21 after induction of anti-GBM GN than wild type (25%)
  • cardiovascular system phenotype
  • altered response to myocardial infarction (J:106065)
    • exhibit worse heart function and greater cardiac dilatation at 35 days after myocardial infarction than wild-type, showing decreased left ventricle end-systolic pressure and left ventricle thickness, increased left ventricle end-systolic volume, heart-to-body weight ratio, septal wall thickness, percentage of left ventricle infracted and collagen content, and significantly different stoke volume, and positive and negative dp/dt
    • wild type bone marrow transplanted into mutant mice rescues the adverse cardiac remodeling and impaired cardiac function after myocardial infarction and shows increased mobilization of angiogenic and natural killer cells
    • increased infarction size (J:106065)
      • 35 days after myocardial infarction show increased percentage of left ventricle infracted
  • decreased vascular permeability (J:84660)
    • do not display increased vascular permeability in the dura mater in response to acute restrain stress as is seen in wild-type controls
  • homeostasis/metabolism phenotype
  • abnormal interferon level (J:131785)
    • treatment with Hrh4 antagonist (JNJ 10191584) during ovalbumin challenge results in decreased levels of IFN-gamma
  • abnormal interleukin level (J:131785)
    • treatment with Hrh4 antagonist (JNJ 10191584) during ovalbumin challenge results in decreased levels of IL-4 (51%), IL-5 (35%), IL-6 (39%), and IL-17 (39%) in cultured peribronchiolar lymph nodes
  • altered response to myocardial infarction (J:106065)
    • exhibit worse heart function and greater cardiac dilatation at 35 days after myocardial infarction than wild-type, showing decreased left ventricle end-systolic pressure and left ventricle thickness, increased left ventricle end-systolic volume, heart-to-body weight ratio, septal wall thickness, percentage of left ventricle infracted and collagen content, and significantly different stoke volume, and positive and negative dp/dt
    • wild type bone marrow transplanted into mutant mice rescues the adverse cardiac remodeling and impaired cardiac function after myocardial infarction and shows increased mobilization of angiogenic and natural killer cells
    • increased infarction size (J:106065)
      • 35 days after myocardial infarction show increased percentage of left ventricle infracted
  • increased porphyrin level (J:5985)
    • moderate but significant increase in protoporphrin levels in red blood cells compared to controls
  • proteinuria (J:113500)
    • mice develop increased proteinuria compared to wild type, 7 and 14 days after induction of anti-glomerular basement membrane (GBM) glomerulonephritis (GN)
  • immune system phenotype
  • *normal* immune system phenotype (J:125656)
    • sensitized mice have a normal early phase reaction (initial phase of bronchoconstriction) after ovalbumin challenge
    • abnormal interferon level (J:131785)
      • treatment with Hrh4 antagonist (JNJ 10191584) during ovalbumin challenge results in decreased levels of IFN-gamma
    • abnormal interleukin level (J:131785)
      • treatment with Hrh4 antagonist (JNJ 10191584) during ovalbumin challenge results in decreased levels of IL-4 (51%), IL-5 (35%), IL-6 (39%), and IL-17 (39%) in cultured peribronchiolar lymph nodes
    • decreased inflammatory response (J:131785)
      • treatment with Hrh4 antagonist (JNJ 10191584) during ovalbumin challenge results in decreased total bronchoalveolar lavage cell numbers (54%) and eosinophils (75%)
    • kidney inflammation (J:113500)
      • more T cells and macrophages infiltrate kidneys compared to control mice; increased numbers of CD4+ T cells and macrophages are found in glomeruli compared to controls on day 14 after anti-GBM GN
  • renal/urinary system phenotype
  • abnormal renal glomerulus morphology (J:113500)
    • in mice there is accumulation of PAS stain-positive material as well as crescent formation in glomeruli, compared to wild type
  • kidney inflammation (J:113500)
    • more T cells and macrophages infiltrate kidneys compared to control mice; increased numbers of CD4+ T cells and macrophages are found in glomeruli compared to controls on day 14 after anti-GBM GN
  • proteinuria (J:113500)
    • mice develop increased proteinuria compared to wild type, 7 and 14 days after induction of anti-glomerular basement membrane (GBM) glomerulonephritis (GN)
  • hematopoietic system phenotype
  • abnormal red blood cell (J:5985)
    • moderate but significant increase in protoporphrin levels in red blood cells compared to controls

The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.

KitW/KitW-v

        involves: C57BL/6J
  • liver/biliary system phenotype
  • hepatic steatosis (J:125831)
    • seen in P7.5 mutants and adults

Gene & Allele Details

Allele Symbol KitW-v
Allele Name viable dominant spotting
Common Name(s) Wv; Wv;
Strain of OriginC57BL
Gene Symbol and Name Kit, kit oncogene
Chromosome 5
Gene Common Name(s) Bs; C-Kit; CD117; Dominant white spotting; Fdc; Gsfsco1; Gsfsco5; Gsfsow3; PBT; SCFR; SCO1; SCO5; SOW3; Ssm; Steel Factor Receptor; Tr-kit; W; belly-spot; dominant spotting; gsf spotted coat 1; gsf spotted coat 5; phenotype like Sl or W 3; spotted sterile male;
Molecular Note A C to T point mutation at nucleotide 2007 results in a threonine to methionine substitution at amino acid 660. [J:24351]
 
Allele Symbol KitW
Allele Name dominant spotting
Common Name(s) W;
Strain of Originold mutant of the mouse fancy
Gene Symbol and Name Kit, kit oncogene
Chromosome 5
Gene Common Name(s) Bs; C-Kit; CD117; Dominant white spotting; Fdc; Gsfsco1; Gsfsco5; Gsfsow3; PBT; SCFR; SCO1; SCO5; SOW3; Ssm; Steel Factor Receptor; Tr-kit; W; belly-spot; dominant spotting; gsf spotted coat 1; gsf spotted coat 5; phenotype like Sl or W 3; spotted sterile male;
General Note This is an old mutant of the mouse fancy. KitW mutants are a potential model for human inherited pure red cell anemia, called Diamond-Blackfan anemia (OMIM 205900), but mouse mutants do not respond to corticosteroid treatment as do human patients. Thus, the mechanism of anemia causation in the two conditions must be different (J:14286).
Molecular Note A guanosine to adenosine substitution at the first nucleotide at the 5' boundary of the intron following the transmembrane exon results in two different aberrantly spliced transcripts putatively expressed in a tissue specific manner. A deletion of 107 bp was found in transcripts from mast cells of mutant mice. A deletion of 234 was found in transcripts from brain or bone marrow cells. The GT to AT point mutation probably disrupted a splice donor site, thereby causing exon skipping. The 107 bp deletion could have resulted from skipping of a transmembrane region exon and the 234 bp deletion from skipping 3 exons. The 107 bp deletion would generate a stop codon 12 bp downstream because of a frame shift, whereas the larger deletion would still be in frame. Northern blot analysis indicated that mast cells from mutants have only 31-37% of the transcripts as mast cells derived from normal bone marrow, suggesting that the mutation may reduce efficiency and authenticity of transcription and splicing. [J:91867]
 
Allele Symbol Pde6brd1
Allele Name retinal degeneration 1
Common Name(s) rd; rd-1; rd1; rodless retina;

Control Information

  Control
   +/+ from the colony
 
  Considerations for Choosing Controls

Colony Maintenance

Diet Information LabDiet® 5K52/5K67

Related Strains

Strains carrying   KitW-v allele
000599   B6 x B6CBCa Aw-J/A-T(5;13)264Ca KitW-v/J
000194   B6.Cg-Lx KitW-v/J
000350   B6By.Cg-KitW-v MitfMi-wh T/J
000049   C57BL/6J-KitW-v/J
View Strains carrying   KitW-v     (4 strains)

Strains carrying   KitW allele
000164   B6.Cg-KitW/J
000092   FL/1Re-KitW/J
000692   WB/ReJ KitW/J
View Strains carrying   KitW     (3 strains)

Strains carrying   Pde6brd1 allele
004202   B6.C3 Pde6brd1 Hps4le/+ +-Lmx1adr-8J/J
000002   B6.C3-Pde6brd1 Hps4le/J
001022   B6C3FeF1/J a/a
000652   BDP/J
000653   BUB/BnJ
002439   C3.129P2(B6)-B2mtm1Unc/J
005494   C3.129S1(B6)-Grm1rcw/J
000480   C3.MRL-Faslpr/J
001957   C3A Pde6brd1.O20/A-Prph2Rd2/J
005973   C3Bir.129P2(B6)-Il10C3Bir/LtJ
004326   C3Bir.129P2(B6)-Il10tm1Cgn/Lt
003968   C3Bir.129P2(B6)-Il10tm1Cgn/LtJ
001906   C3Ga.Cg-Catb/J
001904   C3H-Atcayji-hes/J
000659   C3H/HeJ
000784   C3H/HeJ-Faslgld/J
000509   C3H/HeJ-Lystbg-2J/J
002433   C3H/HeJ-Spnb4qv-lnd2J/J
005972   C3H/HeJBirLtJ
001824   C3H/HeJSxJ
000635   C3H/HeOuJ
000474   C3H/HeSn
001431   C3H/HeSn-ocd/J
000661   C3H/HeSnJ
002235   C3H/HeSnJ-Ctnna2cdf/J
002333   C3H/HeSnJ-gri/J
006435   C3HeB.SW-Soaa/MonJ
000658   C3HeB/FeJ
001576   C3HeB/FeJ-Atp7btx-J/J
002588   C3HeB/FeJ-Eya1bor/J
001533   C3HeB/FeJ-Mc1rE-so Gli3Xt-J/J
001886   C3HeB/FeJLe a/a-gnd/J
001908   C3HfB/BiJ
001502   C3Sn.B6-Epha4rb/J
001547   C3Sn.Cg-Cm/J
000656   CBA/J
000813   CBA/J-Atp7aMo-pew/J
000660   DA/HuSnJ
000023   FL/1ReJ
000025   FL/4ReJ
003078   FVB-Tg(WapIgf1)39Dlr/J
003024   FVB.129P2(B6)-Fmr1tm1Cgr/J
002539   FVB.129P2-Abcb4tm1Bor/J
002935   FVB.129S2(B6)-Ccnd1tm1Wbg/J
002953   FVB.Cg-Tg(MMTVTGFA)254Rjc/J
003170   FVB.Cg-Tg(Myh6-tTA)6Smbf/J
003257   FVB/N-Tg(GFAPGFP)14Mes/J
002374   FVB/N-Tg(MMTV-PyVT)634Mul/J
002856   FVB/N-Tg(TIE2-lacZ)182Sato/J
002384   FVB/N-Tg(UcpDta)1Kz/J
001800   FVB/NJ
003487   FVB/NJ-Tg(XGFAP-lacZ)3Mes/J
001491   FVB/NMob
000734   MOLD/RkJ
000550   MOLF/EiJ
002423   NON/ShiLtJ
000679   P/J
000680   PL/J
100299   PLSJLF1/J
000269   SB/LeJ
005651   SJL.AK-Thy1a/TseJ
000686   SJL/J
000688   ST/bJ
004808   STOCK Mapttm1(EGFP)Klt Tg(MAPT)8cPdav/J
002648   STOCK a/a Cln6nclf/J
000279   STOCK gr +/+ Ap3d1mh/J
005965   STOCK Tg(Pomc1-cre)16Lowl/J
004770   SW.B6-Soab/J
002023   SWR.M-Emv21 Emv22/J
000689   SWR/J
000939   SWR/J-Clcn1adr-mto/J
000692   WB/ReJ KitW/J
000693   WC/ReJ KitlSl/J
100401   WCB6F1/J KitlSl KitlSl-d
View Strains carrying   Pde6brd1     (74 strains)

View Strains carrying other alleles of Kit     (29 strains)

View Strains carrying other alleles of Pde6b     (8 strains)

Additional Web Information

Genetic Quality Control Annual Report
JAX Notes, Winter 1991; 444. Coat Colors of Anemic Mice.

Animal Health Reports

Room Number           AX5

Research Applications

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

Hematological Research
Anemia, Iron Deficiency and Transport Defects
Mast Cell Deficiency

Internal/Organ Research
Gastrointestinal Defects

KitW-v related

Cancer Research
Growth Factors/Receptors/Cytokines
Increased Tumor Incidence (Gonadal Tumors: ovarian)
Oncogenes

Dermatology Research
Color and White Spotting Defects

Developmental Biology Research
Neural Crest Defects

Endocrine Deficiency Research
Bone/Bone Marrow Defects
Gonad Defects
Skin Defects

Immunology and Inflammation Research
Immunodeficiency (Mast Cell Deficiency)

Mouse/Human Gene Homologs
piebaldism
synpolydactyly

Neurobiology Research
Receptor Defects
Vestibular and Hearing Defects

Reproductive Biology Research
Developmental Defects Affecting Gonads (germ cell deficient)
Fertility Defects
Gonadal Tumors (ovarian)

Research Tools
Immunology and Inflammation Research (Mast Cell Deficiency)

Sensorineural Research
Vestibular and Hearing Defects

KitW related

Cancer Research
Growth Factors/Receptors/Cytokines
Increased Tumor Incidence (Gonadal Tumors: ovarian)
Oncogenes

Dermatology Research
Color and White Spotting Defects

Developmental Biology Research
Neural Crest Defects

Endocrine Deficiency Research
Bone/Bone Marrow Defects
Gonad Defects
Skin Defects

Immunology and Inflammation Research
Immunodeficiency (Mast Cell Deficiency)

Mouse/Human Gene Homologs
piebaldism
synpolydactyly

Neurobiology Research
Receptor Defects
Vestibular and Hearing Defects

Reproductive Biology Research
Developmental Defects Affecting Gonads (germ cell deficient)
Fertility Defects
Gonadal Tumors (ovarian)

Research Tools
Immunology and Inflammation Research (Mast Cell Deficiency)

Sensorineural Research
Retinal Degeneration
Vestibular and Hearing Defects

Pde6brd1 related

Mouse/Human Gene Homologs
retinitis pigmentosa, autosomal recessive

Sensorineural Research
Retinal Degeneration

References

Selected Reference(s)

Arguello F; Furlanetto RW; Baggs RB; Graves BT; Harwell SE; Cohen HJ; Frantz CN. 1992. Incidence and distribution of experimental metastases in mutant mice with defective organ microenvironments (genotypes Sl/Sld and W/Wv). Cancer Res 52(8):2304-9. [PubMed: 1559233]  [J:468]

Murphy ED. 1972. Hyperplastic and early neoplastic changes in the ovaries of mice after genic deletion of germ cells. J Natl Cancer Inst 48(5):1283-95. [PubMed: 4337905]  [J:5274]

Nocka K; Tan JC; Chiu E; Chu TY; Ray P; Traktman P; Besmer P. 1990. Molecular bases of dominant negative and loss of function mutations at the murine c-kit/white spotting locus: W37, Wv, W41 and W. EMBO J 9(6):1805-13. [PubMed: 1693331]  [J:10528]

Reis MM; Tsai MC; Schlegel PN; Feliciano M; Raffaelli R; Rosenwaks Z; Palermo GD. 2000. Xenogeneic transplantation of human spermatogonia. Zygote 8(2):97-105. [PubMed: 10857580]  [J:109890]

Shyu H; Hsu S; Hsieh-Li H; Li H. 2001. A novel member of the RBCC family, Trif, expressed specifically in the spermatids of mouse testis. Mech Dev 108(1-2):213-6. [PubMed: 11578878]  [J:71914]

Additional References

Price and Supply Information

Strain Name: WBB6F1/J-KitW/KitW-v/J
Stock Number: 100410

Price Details

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Supply Details

Standard SupplyLevel 2. Up to 100 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.
Genomic DNA is available for this strain from the Mouse DNA Resource.
LicensingSee General Terms and Conditions below  
Control InformationView Control Information in Strain Details.

General Terms and Conditions

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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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