Strain Name:

C57BL/6J-Pou4f3ddl/J

Stock Number:

003484

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Availability:

Cryopreserved - Ready for recovery

Description

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Strain Information

Type Mutant Strain; Spontaneous Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Specieslaboratory mouse

Appearance
black, circling
Related Genotype: a/a Pou4f3ddl/Pou4f3ddl

black, unaffected
Related Genotype: a/a Pou4f3ddl/+

Control Information

  Control
   Heterozygote from the colony
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Related Strains

Strains carrying other alleles of Pou4f3
010560   B6.129-Pou4f3tm1.1Nat/J
008645   B6;129S7-Pou4f3tm1Xia/J
View Strains carrying other alleles of Pou4f3     (2 strains)

Phenotype

Phenotype Information

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 Dominant 15; DFNA15   (POU4F3)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Pou4f3ddl/Pou4f3ddl

        C57BL/6J-Pou4f3ddl/J
  • behavior/neurological phenotype
  • absent pinna reflex
    • homozygotes show no Preyer reflex   (MGI Ref ID J:55448)
  • circling   (MGI Ref ID J:55448)
  • head tossing   (MGI Ref ID J:92833)
    • homozygotes display vertical head tossing   (MGI Ref ID J:55448)
  • hyperactivity   (MGI Ref ID J:92833)
    • homozygotes exhibit a general hyperactive behavior typical of the shaker-waltzer class of mutants   (MGI Ref ID J:55448)
  • hearing/vestibular/ear phenotype
  • abnormal cochlear outer hair cell morphology
    • at E18.5, OHC morphology and patterning is markedly abnormal   (MGI Ref ID J:92833)
    • abnormal outer hair cell stereociliary bundle morphology
      • at E18.5, no intact OHC stereociliary bundles can be identified with phalloidin staining   (MGI Ref ID J:92833)
      • at P0, long and aberrant stereocilia-like structures are identified on the surface of some degenerating OHCs   (MGI Ref ID J:92833)
    • cochlear outer hair cell degeneration
      • at E18.5, homozygotes exhibit OHC degeneration in the base and apex of the cochlea   (MGI Ref ID J:92833)
      • less severe OHC degeneration is noted at E16.5   (MGI Ref ID J:92833)
  • cochlear hair cell degeneration
    • at P0, most of the basal sensory epithelium has degenerated   (MGI Ref ID J:92833)
    • cochlear inner hair cell degeneration
      • at E18.5, IHCs are readily identified in the apex but are clearly absent from the most basal 30-35% of the cochlear duct   (MGI Ref ID J:92833)
      • less severe IHC degeneration is noted at E16.5   (MGI Ref ID J:92833)
      • at P0, no IHCs can be identified in the basal parts   (MGI Ref ID J:92833)
    • cochlear outer hair cell degeneration
      • at E18.5, homozygotes exhibit OHC degeneration in the base and apex of the cochlea   (MGI Ref ID J:92833)
      • less severe OHC degeneration is noted at E16.5   (MGI Ref ID J:92833)
  • deafness   (MGI Ref ID J:92833)
    • homozygotes are deaf; however, the anatomy of the inner ear is grossly normal   (MGI Ref ID J:55448)
  • increased or absent threshold for auditory brainstem response
    • homozygotes show no auditory brainstem responses up to 99 dB SPL   (MGI Ref ID J:55448)
    • homozygotes exhibit increased auditory brainstem response (ABR) thresholds (>100 db SPL) relative to age-matched wild-type and heterozygous mice (35 db SPL)   (MGI Ref ID J:92833)
  • pillar cell degeneration
    • at E18.5, a slow degeneration of pillar cells occurs in a wave that begins from the base of the cochlea toward the apex   (MGI Ref ID J:92833)
    • at this stage, an undisrupted row of pillar cells is clearly present in the apex   (MGI Ref ID J:92833)
  • growth/size/body phenotype
  • decreased body size
    • affected homozygotes are slightly smaller than wild-type littermates   (MGI Ref ID J:55448)
  • nervous system phenotype
  • abnormal cochlear outer hair cell morphology
    • at E18.5, OHC morphology and patterning is markedly abnormal   (MGI Ref ID J:92833)
    • abnormal outer hair cell stereociliary bundle morphology
      • at E18.5, no intact OHC stereociliary bundles can be identified with phalloidin staining   (MGI Ref ID J:92833)
      • at P0, long and aberrant stereocilia-like structures are identified on the surface of some degenerating OHCs   (MGI Ref ID J:92833)
    • cochlear outer hair cell degeneration
      • at E18.5, homozygotes exhibit OHC degeneration in the base and apex of the cochlea   (MGI Ref ID J:92833)
      • less severe OHC degeneration is noted at E16.5   (MGI Ref ID J:92833)
  • cochlear hair cell degeneration
    • at P0, most of the basal sensory epithelium has degenerated   (MGI Ref ID J:92833)
    • cochlear inner hair cell degeneration
      • at E18.5, IHCs are readily identified in the apex but are clearly absent from the most basal 30-35% of the cochlear duct   (MGI Ref ID J:92833)
      • less severe IHC degeneration is noted at E16.5   (MGI Ref ID J:92833)
      • at P0, no IHCs can be identified in the basal parts   (MGI Ref ID J:92833)
    • cochlear outer hair cell degeneration
      • at E18.5, homozygotes exhibit OHC degeneration in the base and apex of the cochlea   (MGI Ref ID J:92833)
      • less severe OHC degeneration is noted at E16.5   (MGI Ref ID J:92833)
View Research Applications

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

Pou4f3ddl related

Neurobiology Research
Hearing Defects
Vestibular Defects

Sensorineural Research
Hearing Defects
Vestibular Defects

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Pou4f3ddl
Allele Name dreidel
Allele Type Spontaneous
Common Name(s) ddl;
Strain of OriginC57BL/6J
Gene Symbol and Name Pou4f3, POU domain, class 4, transcription factor 3
Chromosome 18
Gene Common Name(s) BRN3C; Brn-3.1; Brn3.1; Brn3c; Brn3c POU domain transcription factor; DFNA15; ddl; dreidel;
Molecular Note The molecular mutation of this allele is a TG dinucleotide deletion (nt #841-842 from GenBank accession number S69352) resulting in premature stop codon upstream of the POU box and homeodomains of the encoded protein. [MGI Ref ID J:55448]

Genotyping

Genotyping Information


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Additional References

Pou4f3ddl related

Frankel WN; Mahaffey CL; Bartlett FS 2nd. 1999. The mutant mouse dreidel (ddl) contains a mutation in the Pou4f3 gene Personal Communication :.  [MGI Ref ID J:55448]

Hertzano R; Dror AA; Montcouquiol M; Ahmed ZM; Ellsworth B; Camper S; Friedman TB; Kelley MW; Avraham KB. 2007. Lhx3, a LIM domain transcription factor, is regulated by Pou4f3 in the auditory but not in the vestibular system. Eur J Neurosci 25(4):999-1005. [PubMed: 17331196]  [MGI Ref ID J:119779]

Hertzano R; Montcouquiol M; Rashi-Elkeles S; Elkon R; Yucel R; Frankel WN; Rechavi G; Moroy T; Friedman TB; Kelley MW; Avraham KB. 2004. Transcription profiling of inner ears from Pou4f3(ddl/ddl) identifies Gfi1 as a target of the Pou4f3 deafness gene. Hum Mol Genet 13(18):2143-53. [PubMed: 15254021]  [MGI Ref ID J:92833]

Weston MD; Pierce ML; Jensen-Smith HC; Fritzsch B; Rocha-Sanchez S; Beisel KW; Soukup GA. 2011. MicroRNA-183 family expression in hair cell development and requirement of microRNAs for hair cell maintenance and survival. Dev Dyn 240(4):808-19. [PubMed: 21360794]  [MGI Ref ID J:169673]

Health & husbandry

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Health & Colony Maintenance Information

Animal Health Reports

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200.

Colony Maintenance

Breeding & Husbandryhomozygous females do not make good mothers.

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


Pricing for USA, Canada and Mexico shipping destinations View International Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $3300.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery - Standard.
    Progeny testing is not required.

    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. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $4290.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery - Standard.
    Progeny testing is not required.

    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. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

General Supply Notes

  • View the complete collection of spontaneous mutants in the Mouse Mutant Resource.

Control Information

  Control
   Heterozygote from the colony
   000664 C57BL/6J
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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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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