Description

Strain Information

Former Names B6.Cg-wi Tyrp1b/+ +/J    (Changed: 15-DEC-04 ) Type Congenic; Mutant Strain; Additional information on Genetically Engineered Mutant Mice. Species laboratory mouse Background Strain C57BL/6J Donor Strain Whrnwi , Holman's recessive stock; Tyrp1b , Holman's recessive stock Generation N16p

Description
At 9 to 10 days of age Whrn^wi homozygotes display increased activity, decreased ability to right their selves, and are generally smaller than unaffected siblings. By 14 to 16 days of age homozygotes display head bobbing, circling, and an unsteady gate. Adults display head tossing and circling behavior and deafness is indicated by 20 days of age. Sackler and Weltman reported the average number of circles by a prodded 12-week old female homozygote to be 407 in 10 minutes. They found female homozygotes to have an increased metabolic rate and adrenocorticosteroid activity and decreased body weights. Homozygous males were reported to have decreased blood glucose and liver glycogen levels, decreased white blood cell and eosinophil counts, increased plasma albumin levels, and decreased globulin levels, in addition to decreased weight and increased food intake. (Lane 1963; Sackler and Weltman 1967, 1970, 1971.)

While the organ of Corti in whirler homozygotes shows normal gross cellular architecture at postnatal day (P) 35, stereocilia are shorter and stubbier. The inner hair cells have shorter stereocila as early as embryonic day (E) 18.5, are less than half the length of wild type by P5, and the normal graded variation in length and width along stereocilia ranks is diminished. While normal outer hair cell stereocillia develop a W-shaped array of ranks with a basal to apical increase in length, the outer hair cell stereocilia in whirler homozygotes develop a U-shaped or V-shaped array and the variation in length is diminished. The outer hair cells begin to degenerate by P60 while inner hair cells take longer to show degeneration, but both are undergoing degeneration by P80. (Kiernan et al., 1998; Holme et al., 2002.)

Development
The wi mutation arose spontaneously in approximately 1955 in a multiple recessive stock homozygous for a, Tyrp1^b, Myo5a^d, Oca2^p, and Ednrb^s in the laboratory of Dr. Meredith N. Runner. This was the HO stock received from Dr. Holman. The phenotype was initially identified in a single male that was then outcrossed to C57BL/6J and the offspring were intercrossed once, non-sibling intercrossed once, then sibling mated to F5 before being maintained by backcross-intercross to C57BL/6J until N7. They were then maintained primarily by sibling intercross with occasional backcross to a parent until they were transferred to Dr. Eva Eicher in 1971. This strain has been maintained with Whrn^wi in coupling with Tyrp1^b from the original stock background on which the Whrn^wi mutation arose. In 1981 N15F8 Whrn^wi Tyrp1^b/Whrn^wi Tyrp1^b males were bred with C57BL/6J females to generate embryos for cryopreservation.

Related Strains

Strains carrying   Tyrp1^b allele

000004	ABP/LeJ
000027	B6.D-Tyrp1b m/J
000670	DBA/1J
000265	MY/HuLeJ
001045	SI/Col Tyrp1b Dnahc11iv/J
000064	STOCK a Tyrp1b Sisi/J
002238	STOCK a Tyrp1b shmy/J
001432	STOCK a/a Tyrp1b sks/Tyrp1b +/J
000594	STOCK T(2;8)26H a/T(2;8)26H a Tyrp1+/Tyrp1b/J
001101	STOCK T(3;4)5Rk Tyrp1b/J
000274	TSJ/LeJ

View Strains carrying   Tyrp1^b     (11 strains)

Strains carrying other alleles of Tyrp1

000068	C57BL/6J-Tyrp1b-J/J
000093	C57BL/6J-Tyrp1b-cJ/J
000671	DBA/2J
003588	LT/SvEi
006252	LT/SvEiJ
002142	STOCK 11R30m/J
000594	STOCK T(2;8)26H a/T(2;8)26H a Tyrp1+/Tyrp1b/J

View Strains carrying other alleles of Tyrp1     (7 strains)

Additional Web Information

Congenic Nomenclature

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms

Deafness, Autosomal Recessive 31; DFNB31 - Models with phenotypic similarity to human disease where etiologies involve orthologs.1

1 Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).

View Mammalian Phenotype Terms

Mammalian Phenotype Terms

      assigned by genotype

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

Whrn^wi/Whrn⁺

        involves: C57BL/6J * STOCK a Tyrp1^b Myo5a^d Oca2^p Ednrb^s

• hearing/vestibular/ear phenotype
• abnormal cochlear inner hair cell morphology (MGI Ref ID J:77939)
• short inner hair cell stereocilia (MGI Ref ID J:77939)
  • the stereocilia of inner hair cells in heterozygotes are slightly shorter at P35
  • by P80 no hair cells are degenerating in the cochlear duct of heterozygotes unlike in homozygotes
• nervous system phenotype
• abnormal cochlear inner hair cell morphology (MGI Ref ID J:77939)
• short inner hair cell stereocilia (MGI Ref ID J:77939)
  • the stereocilia of inner hair cells in heterozygotes are slightly shorter at P35
  • by P80 no hair cells are degenerating in the cochlear duct of heterozygotes unlike in homozygotes

Whrn^wi/Whrn^wi

        involves: C57BL/6J * STOCK a Tyrp1^b Myo5a^d Oca2^p Ednrb^s

• adipose tissue phenotype
• decreased adipose tissue amount (MGI Ref ID J:5538)
  • there is a noticeable deficiency of adipose tissue in the abdominal cavity
• behavior/neurological phenotype
• abnormal gait (MGI Ref ID J:269)
  • at P14 to P16 the gait is unsteady and mutants constantly topple over
• abnormal maternal nurturing (MGI Ref ID J:269)
  • females tend to trample their young
• circling (MGI Ref ID J:269)
• head bobbing (MGI Ref ID J:48657)
  • this behavior is noticeable at P14 to P16
• head tossing (MGI Ref ID J:5037)
  • this behavior is seen in adults
• hyperactivity (MGI Ref ID J:5037)
  • mutants are restless and excitable
• impaired righting response (MGI Ref ID J:269)
  • at P9 to P10 when placed on their backs mutants take longer to return to an upright position
• impaired swimming (MGI Ref ID J:269)
  • some mutants at weaning age can swim if the head is not submerged
  • adults can not swim
• cardiovascular system phenotype
• increased heart weight (MGI Ref ID J:5538)
  • the heart weight at 16 weeks in male mutants is significantly larger
• endocrine/exocrine gland phenotype
• decreased seminal gland weight (MGI Ref ID J:5538)
  • the seminal vesicle weight at 16 weeks in male mutants is significantly smaller
• enlarged adrenal glands (MGI Ref ID J:5037)
  • the adrenal weight at 3.5, 10, and 18 months in female mutants is significantly higher
  • the adrenal weight at 16 weeks in male mutants is significantly higher
• growth/size phenotype
• decreased body weight (MGI Ref ID J:269)
  • at P9 to P10 and P14 to P16 mutants are smaller
  • the body weight at 3.5 and 18 months in female mutants is significantly lower
  • the body weight at 13 to 16 weeks in male mutants is significantly lower
• hearing/vestibular/ear phenotype
• abnormal cochlear hair cell stereociliary bundle morphology (MGI Ref ID J:77939)
  • the specialized microvilli (stereocilia) that project from the apical surface of the inner and outer hair cells are abnormal
• abnormal outer hair cell stereociliary bundle morphology (MGI Ref ID J:77939)
  • outer hair cells appear normal until P4
  • on P4 the stereocilia are arranged in a U-shaped pattern rather than the normal W-shaped pattern
  • in mutants on P15 and P35 stereocilia height within a rank of outer hair cells is irregular instead of uniform
  • an increase in diameter and variable heights within bundles
  • at P5 (but not at P3), the center of the developing W-shape of OHC stereocilia still contains excess microvilli which have not yet been absorbed, indicating delayed development of OHC stereociliary bundles
  • at P10, the extra OHC microvilli in the center of the W-shape have not yet cleared totally in the apical turn but have disappeared in the basal turn, while kinocilia are still present but regress by P15
• decreased outer hair cell stereocilia number (MGI Ref ID J:122600)
  • the number of outer hair cell stereocilia was sgnificantly reduced
  • the center-to-center spacing between the stereocilia was greater
• short inner hair cell stereocilia (MGI Ref ID J:77939)
  • overall the stereocilia of inner hair cells in mutants are significantly shorter at E18.5, P1, and P35
  • the length of stereocilia on inner hair cells in mutants decreases between P1 and P4 and between P4 and P35 rather than increasing
  • the morphological differences between stereocilia in different ranks of inner hair cells are not as prominent in mutants at P35
  • however in mutants the stereocilia at the center of a rank are still significantly taller than those on the edge as is seen in controls
  • stereocilia of inner hair cells are short with larger diameters without a corresponding increase in the number of actin filaments at P20
  • from P5 onwards, IHCs are abnormal with many short, stubby stereocilia, although the kinocilium is correctly positioned and of normal length
• abnormal cochlear inner hair cell morphology (MGI Ref ID J:48657)
  • from P5 up to P40, all IHCs along the entire length of the cochlea are similarly affected, with many abnormal stereocilia
• cochlear inner hair cell degeneration (MGI Ref ID J:77939)
  • by P80 both outer hair cells and inner hair cells in the base of the cochlear duct are degenerating
  • at P60, IHCs have still not degenerated or developed further; however, IHCs are degenerating at P80
• short inner hair cell stereocilia (MGI Ref ID J:77939)
  • overall the stereocilia of inner hair cells in mutants are significantly shorter at E18.5, P1, and P35
  • the length of stereocilia on inner hair cells in mutants decreases between P1 and P4 and between P4 and P35 rather than increasing
  • the morphological differences between stereocilia in different ranks of inner hair cells are not as prominent in mutants at P35
  • however in mutants the stereocilia at the center of a rank are still significantly taller than those on the edge as is seen in controls
  • stereocilia of inner hair cells are short with larger diameters without a corresponding increase in the number of actin filaments at P20
  • from P5 onwards, IHCs are abnormal with many short, stubby stereocilia, although the kinocilium is correctly positioned and of normal length
• circling (MGI Ref ID J:269)
• cochlear hair cell degeneration (MGI Ref ID J:48657)
  • at P80, both IHCs and OHCs are degenerating
• cochlear outer hair cell degeneration (MGI Ref ID J:77939)
  • by P60 outer hair cells are showing signs of degeneration
  • by P80 both outer hair cells and inner hair cells in the base of the cochlear duct are degenerating
  • by P80 only outer hair cells are degenerating in the apex of the cochlear duct
  • although apparently normal at P15, OHCs start showing signs of degeneration from P60 onwards
• deafness (MGI Ref ID J:48657)
  • adults are deaf
  • adults are deaf
• head bobbing (MGI Ref ID J:48657)
  • this behavior is noticeable at P14 to P16
• head tossing (MGI Ref ID J:5037)
  • this behavior is seen in adults
• homeostasis/metabolism phenotype
• decreased circulating glucose level (MGI Ref ID J:5538)
  • the blood glucose levels are significantly lower at 16 weeks in male mutants
• decreased glycogen level (MGI Ref ID J:5538)
  • liver glycogen and liver phosphorylase levels are significantly lower at 16 weeks in male mutants
• increased circulating corticosterone level (MGI Ref ID J:5538)
  • consistent and significantly higher corticosterone levels in the plasma and adrenals
  • corticosterone levels are higher in the adrenals when comparing per pair of adrenals or per 100 mg adrenals
• increased oxygen consumption (MGI Ref ID J:5037)
  • free and semi-restrained O₂ consumption is increased at 12 and 14 weeks and 14 months
  • free and semi-restrained O₂ consumption is increased at 13 and 14 weeks respectively
• immune system phenotype
• decreased leukocyte cell number (MGI Ref ID J:5538)
  • the white blood cell counts are significantly smaller at 15 weeks in male mutants
• decreased thymus weight (MGI Ref ID J:5037)
  • the thymus weight at 3.5 and 18 months in female mutants is significantly lower
• liver/biliary system phenotype
• increased liver weight (MGI Ref ID J:5037)
  • the liver weight at 3.5 and 10 months in female mutants is significantly higher
  • this difference is no longer significant at 18 months
• renal/urinary system phenotype
• increased kidney weight (MGI Ref ID J:5037)
  • the kidney weight at 3.5 and 10 months in female mutants is significantly higher
  • this difference is no longer significant at 18 months
• reproductive system phenotype
• decreased seminal gland weight (MGI Ref ID J:5538)
  • the seminal vesicle weight at 16 weeks in male mutants is significantly smaller
• decreased uterus weight (MGI Ref ID J:5037)
  • the uterine weight at 3.5 months in female mutants is significantly smaller
  • there is no significant difference at 10 and 18 months
• nervous system phenotype
• abnormal cochlear hair cell stereociliary bundle morphology (MGI Ref ID J:77939)
  • the specialized microvilli (stereocilia) that project from the apical surface of the inner and outer hair cells are abnormal
• abnormal outer hair cell stereociliary bundle morphology (MGI Ref ID J:77939)
  • outer hair cells appear normal until P4
  • on P4 the stereocilia are arranged in a U-shaped pattern rather than the normal W-shaped pattern
  • in mutants on P15 and P35 stereocilia height within a rank of outer hair cells is irregular instead of uniform
  • an increase in diameter and variable heights within bundles
  • at P5 (but not at P3), the center of the developing W-shape of OHC stereocilia still contains excess microvilli which have not yet been absorbed, indicating delayed development of OHC stereociliary bundles
  • at P10, the extra OHC microvilli in the center of the W-shape have not yet cleared totally in the apical turn but have disappeared in the basal turn, while kinocilia are still present but regress by P15
• decreased outer hair cell stereocilia number (MGI Ref ID J:122600)
  • the number of outer hair cell stereocilia was sgnificantly reduced
  • the center-to-center spacing between the stereocilia was greater
• short inner hair cell stereocilia (MGI Ref ID J:77939)
  • overall the stereocilia of inner hair cells in mutants are significantly shorter at E18.5, P1, and P35
  • the length of stereocilia on inner hair cells in mutants decreases between P1 and P4 and between P4 and P35 rather than increasing
  • the morphological differences between stereocilia in different ranks of inner hair cells are not as prominent in mutants at P35
  • however in mutants the stereocilia at the center of a rank are still significantly taller than those on the edge as is seen in controls
  • stereocilia of inner hair cells are short with larger diameters without a corresponding increase in the number of actin filaments at P20
  • from P5 onwards, IHCs are abnormal with many short, stubby stereocilia, although the kinocilium is correctly positioned and of normal length
• abnormal cochlear inner hair cell morphology (MGI Ref ID J:48657)
  • from P5 up to P40, all IHCs along the entire length of the cochlea are similarly affected, with many abnormal stereocilia
• cochlear inner hair cell degeneration (MGI Ref ID J:77939)
  • by P80 both outer hair cells and inner hair cells in the base of the cochlear duct are degenerating
  • at P60, IHCs have still not degenerated or developed further; however, IHCs are degenerating at P80
• short inner hair cell stereocilia (MGI Ref ID J:77939)
  • overall the stereocilia of inner hair cells in mutants are significantly shorter at E18.5, P1, and P35
  • the length of stereocilia on inner hair cells in mutants decreases between P1 and P4 and between P4 and P35 rather than increasing
  • the morphological differences between stereocilia in different ranks of inner hair cells are not as prominent in mutants at P35
  • however in mutants the stereocilia at the center of a rank are still significantly taller than those on the edge as is seen in controls
  • stereocilia of inner hair cells are short with larger diameters without a corresponding increase in the number of actin filaments at P20
  • from P5 onwards, IHCs are abnormal with many short, stubby stereocilia, although the kinocilium is correctly positioned and of normal length
• cochlear hair cell degeneration (MGI Ref ID J:48657)
  • at P80, both IHCs and OHCs are degenerating
• cochlear outer hair cell degeneration (MGI Ref ID J:77939)
  • by P60 outer hair cells are showing signs of degeneration
  • by P80 both outer hair cells and inner hair cells in the base of the cochlear duct are degenerating
  • by P80 only outer hair cells are degenerating in the apex of the cochlear duct
  • although apparently normal at P15, OHCs start showing signs of degeneration from P60 onwards
• hematopoietic system phenotype
• decreased leukocyte cell number (MGI Ref ID J:5538)
  • the white blood cell counts are significantly smaller at 15 weeks in male mutants
• decreased thymus weight (MGI Ref ID J:5037)
  • the thymus weight at 3.5 and 18 months in female mutants is significantly lower

Whrn^wi/Whrn^wi

        involves: STOCK a Tyrp1^b Myo5a^d Oca2^p Ednrb^s

• hearing/vestibular/ear phenotype
• abnormal cochlear hair cell stereociliary bundle morphology (MGI Ref ID J:132633)
  • hair cell stereocilia are shorter than in wild-type mice
• nervous system phenotype
• abnormal cochlear hair cell stereociliary bundle morphology (MGI Ref ID J:132633)
  • hair cell stereocilia are shorter than in wild-type mice

View Research Applications

Research Applications

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

Tyrp1^b related

Dermatology Research
Color and White Spotting Defects

Mouse/Human Gene Homologs
oculocutaneous albinism type III

Whrn^wi related

Metabolism Research
Exertion Related

Neurobiology Research
Vestibular and Hearing Defects

Sensorineural Research
Vestibular and Hearing Defects

Genes & Alleles

Gene & Allele Information

Allele Symbol		Tyrp1b
Allele Name		brown
Allele Type		Spontaneous
Strain of Origin		C57BL
Gene Symbol and Name		Tyrp1, tyrosinase-related protein 1
Chromosome		4
Gene Common Name(s)		B; CAS2; CATB; GP75; TRP; TRP-1; TRP1; TYRP; Tyrp; b; b-PROTEIN; brown; iris stromal atrophy; isa; tyrosinase-related protein;
General Note		Tyrp1b, brown, recessive. This type mutant of the brown locus is an old mutation of the mouse fancy. The eumelanin of the hair and eyes is brown rather than black. The pigment granules also appear brown rather than black and are spheroid rather than ovoid in shape (J:12970). The fine structure of the developing pigment granules is fibrillar, like that of wild-type mice, but the appearance of the mature granule may be more coarsely granular (J:5346, J:5001, J:5068). The granules incorporate twice as much 14C-tyrosine as normal (J:12173).
Molecular Note		A G-to-A transition point mutation at position 329 was shown by revertant analysis to be responsible for the mutant phenotype seen in the brown mutant. This mutation is predicted to change a cysteine residue to a tyrosine in the encoded protein. Three other point mutations in the brown sequence were identified, but do not contribute to the mutant phenotype. [MGI Ref ID J:44435]
Allele Symbol		Whrnwi
Allele Name		whirler
Allele Type		Spontaneous
Common Name(s)		wi;
Strain of Origin		STOCK a Tyrp1 Myo5a Oca2 Ednrb
Gene Symbol and Name		Whrn, whirlin
Chromosome		4
Gene Common Name(s)		1110035G07Rik; AW122018; AW742671; C430046P22Rik; CIP98; DKFZp434N014; KIAA1526; RIKEN cDNA 1110035G07 gene; RIKEN cDNA C430046P22 gene; RP11-9M16.1; USH2D; WI; expressed sequence AW122018; expressed sequence AW742671; mKIAA1526; whirler; wi;
General Note		Viability of homozygotes may be slightly reduced. Both sexes are fertile, but females do not always make good mothers (J:269). They have defects of the membranous labyrinth similar to those of Myo15 (M.S. Deol, personal communication).
Molecular Note		The mutation was identified as a 526 bp deletion encompassing the first putative methionine of the short C-terminal isoform and part of the long isoform. This creates a frameshift resulting in premature termination of the long isoform before the third PDZ domain. [MGI Ref ID J:86904]

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)

Lane PW. 1963. Whirler mice, a recessive behavior mutation in linkage group VIII J Hered 54:263-6. [PubMed: 14098314]  [MGI Ref ID J:269]

Additional References

Fleming J; Rogers MJ; Brown SD; Steel KP. 1994. Linkage analysis of the whirler deafness gene on mouse chromosome 4. Genomics 21(1):42-8. [PubMed: 8088814]  [MGI Ref ID J:18269]

Mburu P; Mustapha M; Varela A; Weil D; El-Amraoui A; Holme RH; Rump A; Hardisty RE; Blanchard S; Coimbra RS; Perfettini I; Parkinson N; Mallon AM; Glenister P; Rogers MJ; Paige AJ; Moir L; Clay J; Rosenthal A; Liu XZ; Blanco G; Steel KP; Petit C; Brown SD. 2003. Defects in whirlin, a PDZ domain molecule involved in stereocilia elongation, cause deafness in the whirler mouse and families with DFNB31. Nat Genet 34(4):421-8. [PubMed: 12833159]  [MGI Ref ID J:86904]

Sackler AM; Weltman AS. 1967. Metabolic and endocrine differences between the mutation whirler and normal female mice. J Exp Zool 164(2):133-40. [PubMed: 6034501]  [MGI Ref ID J:5037]

Tyrp1^b related

Anderson MG; Libby RT; Mao M; Cosma IM; Wilson LA; Smith RS; John SW. 2006. Genetic context determines susceptibility to intraocular pressure elevation in a mouse pigmentary glaucoma. BMC Biol 4:20. [PubMed: 16827931]  [MGI Ref ID J:128215]

Anderson MG; Nair KS; Amonoo LA; Mehalow A; Trantow CM; Masli S; John SW. 2008. GpnmbR150X allele must be present in bone marrow derived cells to mediate DBA/2J glaucoma. BMC Genet 9:30. [PubMed: 18402690]  [MGI Ref ID J:134670]

Brooks BP; Larson DM; Chan CC; Kjellstrom S; Smith RS; Crawford MA; Lamoreux L; Huizing M; Hess R; Jiao X; Hejtmancik JF; Maminishkis A; John SW; Bush R; Pavan WJ. 2007. Analysis of ocular hypopigmentation in Rab38cht/cht mice. Invest Ophthalmol Vis Sci 48(9):3905-13. [PubMed: 17724166]  [MGI Ref ID J:124886]

Center EM; Hunter RL; Dodge AH. 1967. Effects of the luxoid gene (lu) on liver esterase isozymes of the mouse. Genetics 55(2):349-58. [PubMed: 6067640]  [MGI Ref ID J:109970]

Coleman DL. 1962. Effect of genic substitution on the incorporation of tyrosine into the melanin of mouse skin. Arch Biochem Biophys 96:562-8. [PubMed: 13880466]  [MGI Ref ID J:12173]

Hearing VJ; Phillips P; Lutzner MA. 1973. The fine structure of melanogenesis in coat color mutants of the mouse. J Ultrastruct Res 43(1):88-106. [PubMed: 4634048]  [MGI Ref ID J:5346]

Howell GR; Libby RT; Jakobs TC; Smith RS; Phalan FC; Barter JW; Barbay JM; Marchant JK; Mahesh N; Porciatti V; Whitmore AV; Masland RH; John SW. 2007. Axons of retinal ganglion cells are insulted in the optic nerve early in DBA/2J glaucoma. J Cell Biol 179(7):1523-37. [PubMed: 18158332]  [MGI Ref ID J:131073]

Hunsicker PR. 1969. White-based brown, B<W> Mouse News Lett 40:41.  [MGI Ref ID J:13492]

Kobayashi T; Imokawa G; Bennett DC; Hearing VJ. 1998. Tyrosinase stabilization by Tyrp1 (the brown locus protein). J Biol Chem 273(48):31801-5. [PubMed: 9822646]  [MGI Ref ID J:51301]

Lamoreux ML; Wakamatsu K; Ito S. 2001. Interaction of major coat color gene functions in mice as studied by chemical analysis of eumelanin and pheomelanin. Pigment Cell Res 14(1):23-31. [PubMed: 11277491]  [MGI Ref ID J:103803]

Markert CL; Silvers WK. 1956. The Effects of Genotype and Cell Environment on Melanoblast Differentiation in the House Mouse. Genetics 41(3):429-50. [PubMed: 17247639]  [MGI Ref ID J:12970]

Matheu A; Pantoja C; Efeyan A; Criado LM; Martin-Caballero J; Flores JM; Klatt P; Serrano M. 2004. Increased gene dosage of Ink4a/Arf results in cancer resistance and normal aging. Genes Dev 18(22):2736-46. [PubMed: 15520276]  [MGI Ref ID J:93879]

Mouse Genome Informatics (MGI). 2005. Information obtained from the Oak Ridge National Laboratory Mutant Mouse Database (ORNL), Oak Ridge, TN (http://bio.lsd.ornl.gov/mouse/) :.  [MGI Ref ID J:100221]

Moyer FH. 1966. Genetic variations in the fine structure and ontogeny of mouse melanin granules. Am Zool 6(1):43-66. [PubMed: 5902512]  [MGI Ref ID J:5001]

Murray WS. 1934. The breeding behavior of the dilute brown stock of mice (Little dba) Am J Cancer 20:573-593.  [MGI Ref ID J:2464]

RIKEN BioResource Center/RIKEN Genomic Sciences Center. 2008. A Large Scale Mutagenesis Program in RIKEN GSC PhenoSITE, World Wide Web (URL: http://www.brc.riken.jp/lab/gsc/mouse/) :.  [MGI Ref ID J:133634]

Raymond S; Jackson IJ. 1994. Molecular characterization of the mouse B<w> mutation causing premature melanocyte death - melanocytes and early development Genet Res 63(2):155 (Abstr).  [MGI Ref ID J:18590]

Rittenhouse E. 1968. Genetic effect on fine structure and development of pigment granules in mouse hair bulb melanocytes. I. The b and d loci. Dev Biol 17(4):351-65. [PubMed: 5650006]  [MGI Ref ID J:5068]

Smyth IM; Wilming L; Lee AW; Taylor MS; Gautier P; Barlow K; Wallis J; Martin S; Glithero R; Phillimore B; Pelan S; Andrew R; Holt K; Taylor R; McLaren S; Burton J; Bailey J; Sims S; Squares J; Plumb B; Joy A; Gibson R; Gilbert J; Hart E; Laird G; Loveland J; Mudge J; Steward C; Swarbreck D; Harrow J; North P; Leaves N; Greystrong J; Coppola M; Manjunath S; Campbell M; Smith M; Strachan G; Tofts C; Boal E; Cobley V; Hunter G; Kimberley C; Thomas D; Cave-Berry L; Weston P; Botcherby MR; White S; Edgar R; C. 2006. Genomic anatomy of the Tyrp1 (brown) deletion complex. Proc Natl Acad Sci U S A 103(10):3704-9. [PubMed: 16505357]  [MGI Ref ID J:107243]

Zdarsky E; Favor J; Jackson IJ. 1990. The molecular basis of brown, an old mouse mutation, and of an induced revertant to wild type. Genetics 126(2):443-9. [PubMed: 2245916]  [MGI Ref ID J:44435]

Whrn^wi related

Belyantseva IA; Boger ET; Naz S; Frolenkov GI; Sellers JR; Ahmed ZM; Griffith AJ; Friedman TB. 2005. Myosin-XVa is required for tip localization of whirlin and differential elongation of hair-cell stereocilia. Nat Cell Biol 7(2):148-56. [PubMed: 15654330]  [MGI Ref ID J:102099]

Holme RH; Kiernan BW; Brown SD; Steel KP. 2002. Elongation of hair cell stereocilia is defective in the mouse mutant whirler. J Comp Neurol 450(1):94-102. [PubMed: 12124769]  [MGI Ref ID J:77939]

Kiernan BW; Mogensen MM; Self TJ; Brown SDM; Steel KP. 1998. Abnormalities of the inner ear in a deaf mouse mutant whirler (wi) Hered Deaf News 15:36-7.  [MGI Ref ID J:48657]

Kikkawa Y; Mburu P; Morse S; Kominami R; Townsend S; Brown SD. 2005. Mutant analysis reveals whirlin as a dynamic organizer in the growing hair cell stereocilium. Hum Mol Genet 14(3):391-400. [PubMed: 15590699]  [MGI Ref ID J:95916]

Mburu P; Kikkawa Y; Townsend S; Romero R; Yonekawa H; Brown SD. 2006. Whirlin complexes with p55 at the stereocilia tip during hair cell development. Proc Natl Acad Sci U S A 103(29):10973-8. [PubMed: 16829577]  [MGI Ref ID J:111803]

Mburu P; Mustapha M; Varela A; Weil D; El-Amraoui A; Holme RH; Rump A; Hardisty RE; Blanchard S; Coimbra RS; Perfettini I; Parkinson N; Mallon AM; Glenister P; Rogers MJ; Paige AJ; Moir L; Clay J; Rosenthal A; Liu XZ; Blanco G; Steel KP; Petit C; Brown SD. 2003. Defects in whirlin, a PDZ domain molecule involved in stereocilia elongation, cause deafness in the whirler mouse and families with DFNB31. Nat Genet 34(4):421-8. [PubMed: 12833159]  [MGI Ref ID J:86904]

Mogensen MM; Rzadzinska A; Steel KP. 2007. The deaf mouse mutant whirler suggests a role for whirlin in actin filament dynamics and stereocilia development. Cell Motil Cytoskeleton 64(7):496-508. [PubMed: 17326148]  [MGI Ref ID J:122600]

Paige AJ; Kiernan BW; Varela A; Rogers MJ; Hughes D; Steel KP; Brown SD. 2000. A deletion on chromosome 4 cosegregates with the whirler deafness mutation: exclusion of Orm1 as a candidate. Mamm Genome 11(1):51-7. [PubMed: 10602993]  [MGI Ref ID J:59252]

Prosser HM; Rzadzinska AK; Steel KP; Bradley A. 2008. Mosaic complementation demonstrates a regulatory role for myosin VIIa in actin dynamics of stereocilia. Mol Cell Biol 28(5):1702-12. [PubMed: 18160714]  [MGI Ref ID J:132633]

Sackler AM; Weltman AS. 1967. Metabolic and endocrine differences between the mutation whirler and normal female mice. J Exp Zool 164(2):133-40. [PubMed: 6034501]  [MGI Ref ID J:5037]

Weltman AS; Sackler AM; Lewis AS; Johnson L. 1970. Metabolism rate, biochemical and endocrine alterations in male whirler mice. Physiol Behav 5(1):17-22. [PubMed: 5538400]  [MGI Ref ID J:5538]

Health & husbandry

Health & Colony Maintenance Information

Currently there no information available for this strain. This may be due to the supply level of this strain.

Purchasing information

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

Pricing

Supply Details

Standard Supply

Repository-Cryopreserved. Must Be Recovered. Please refer to pricing and supply notes for further information.

Supply Notes

Cryorecovery - Standard. The recovery process begins when a signed agreement form is returned to the Customer Service Department after order placement. Although results vary by strain, at least two males and two females (two pairs) will be provided, typically within 15 weeks of our receipt of the signed agreement form. If the first recovery attempt is unsuccessful or only one pair is recovered, a second recovery will be done, extending the delivery time to approximately 25 weeks. At least one member of each pair will be of known genotype and will carry the mutation if it is a mutant strain. Please note that pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation. Price represents a repository maintenance fee, which includes the cost of recovery of the strain from the cryopreservation resource and the periodic replacement of the frozen embryos used for recovery. Cryorecovery to establish a Dedicated Supply for greater quantities of mice. One to two pairs will be recovered to establish a Dedicated Supply of mice. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 or 1-207-288-5845. Genomic DNA is available for this strain from the Mouse DNA Resource.

General Terms and Conditions

See Terms of Use

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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Ordering and Purchasing Information

      Purchasing Information
      JAX^® Mice Orders
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Contact Information
Orders & Technical Support
Tel: 800.422.6423 or 207.288.5845
Fax: 207.288.6150
Technical Support Email Form

Terms of Use

Terms of Use

General Terms and Conditions

Contact information

General inquiries

Contracts Administration

phone:	207-288-6470
fax:	207-288-6655

JAX^® Mice & Services Conditions of Use

“Each recipient institution, including its employees and other researchers under its control (RECIPIENT), of mice or services using mice from The Jackson Laboratory (TJL) agrees that such mice, descendants of those mice derived by inbreeding or crossbreeding, including unmodified derivatives of those mice or their descendants (“MICE”) shall not be: (i) used for any purpose other than the internal research of the RECIPIENT, (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 with respect to MICE. Acceptance of MICE from TJL shall be deemed agreement by RECIPIENT to these conditions, and departure from these conditions requires The Jackson Laboratory’s prior written authorization.”

In case of dissatisfaction for a valid reason and claimed in writing by a purchaser within ninety (90) days of receipt of MICE, products or services, The Jackson Laboratory will, at its option, provide credit or replacement for the MICE or product received or the services provided.

No Liability

In no event shall The Jackson Laboratory, its trustees, directors, officers, employees, and affiliates be liable for any causes of action or damages, including any direct, indirect, special, or consequential damages, arising out of the provision of MICE, products or services, including economic damage or injury to property and lost profits, and including any damage arising from acts or negligence on the part of The Jackson Laboratory, its agents or employees. In purchasing or receiving MICE, products or services from The Jackson Laboratory, purchaser or recipient, or any party claiming by or through them, expressly releases and discharges The Jackson Laboratory from all such causes of action or damages, and further agrees to defend and indemnify The Jackson Laboratory from any costs or damages arising out of any third party claims.

MICE and biological materials are to be used in a safe manner and in accordance with all applicable governmental rules and regulations.

The foregoing represents the General Terms and Conditions applicable to The Jackson Laboratory’s MICE, products and services. In addition, special terms and conditions of sale of certain MICE, products and services may be set forth separately in The Jackson Laboratory 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 The Jackson Laboratory, and by its licensees and distributors.

Acceptance of delivery of MICE, products or services shall be deemed agreement to these terms and conditions. No purchase order or other document transmitted by purchaser or recipient that may modify the terms and conditions hereof, shall be in any way binding on The Jackson Laboratory, and instead the terms and conditions set forth herein, including any special terms and conditions set forth separately, shall govern the sale of MICE, products services by The Jackson Laboratory.