Description

Strain Information

Former Names B6(MOR)-Lhfpl5hscy/J    (Changed: 06-OCT-05 ) B6(MOR)-Tmhshscy/J    (Changed: 29-SEP-05 ) Type Congenic; Mutant Strain; Spontaneous Mutation; Additional information on Genetically Engineered Mutant Mice. Species laboratory mouse Generation +N4F4 (06-DEC-07)

Description
Mice homozygous for the Tmhs^hscy mutation circle rapidly, shake their heads from side to side, fail to swim, and are congenitally deaf. Although hair cells appear normal at birth, they degenerate. At 4 months of age, degeneration of the organ of Corti is also found, more pronounced in the basal region of the cochlea than the apex, with loss of inner and outer hair cells and decreased spiral ganglia (Longo-Guess et al., 2005).

Development
The hurry scurry mutation arose spontaneously in 1992 on the B6.MOR-Gusb^a background in the laboratory of Dr. Gordon Watson at The Jackson Laboratory and was maintained via sibling mating for several generations before being backcrossed three times to C57BL/6, which removed the Gusb^a allele. This strain was subsequently maintained by sibling mating of primarily heterozygous females with homozygous males. In 2005 this strain reached generation N3F15.

Control Information

	Control
	Heterozygote from the colony
Considerations for Choosing Controls

Related Strains

Strains carrying other alleles of Tmhs

005434	B6.129-Tmhstm1Kjn/Kjn
005638	C57BL/6J-Tmhshscy-2J/J

View Strains carrying other alleles of Tmhs     (2 strains)

Phenotype

Phenotype Information

View Mammalian Phenotype Terms

Mammalian Phenotype Terms

      assigned by genotype

Tmhs^hscy/Tmhs^hscy

        B6(MOR)-Tmhs^hscy/J

• behavior/neurological phenotype
• circling (MGI Ref ID J:98396)
• head shaking (MGI Ref ID J:98396)
  • shaking from side to side
• impaired swimming (MGI Ref ID J:98396)
  • inability to swim
• hearing/vestibular/ear phenotype
• absent brainstem auditory evoked potential (MGI Ref ID J:98396)
  • ABR threshold testing showed that no mutant mouse tested exhibited a response to auditory stimuli up to 110 dB sound pressure level
• circling (MGI Ref ID J:98396)
• cochlear hair cell degeneration (MGI Ref ID J:98396)
  • scanning electron microscopy analysis showed that inner and outer hair cells appear normal at birth
• cochlear inner hair cell degeneration (MGI Ref ID J:98396)
  • by P8, inner hair cells appear disorganized with loss of typical cell morphology
  • by P50, the inner hair cells are severely splayed
• cochlear outer hair cell degeneration (MGI Ref ID J:98396)
  • by P8, outer hair cells appear disorganized with loss of typical cell morphology
  • by P50, outer hair cells are absent, with empty patches appearing the basal portion of the cochlea
• deafness (MGI Ref ID J:98396)
• head shaking (MGI Ref ID J:98396)
  • shaking from side to side
• organ of Corti degeneration (MGI Ref ID J:98396)
  • degeneration was more pronounced in the basal region than in the apex
• nervous system phenotype
• cochlear hair cell degeneration (MGI Ref ID J:98396)
  • scanning electron microscopy analysis showed that inner and outer hair cells appear normal at birth
• cochlear inner hair cell degeneration (MGI Ref ID J:98396)
  • by P8, inner hair cells appear disorganized with loss of typical cell morphology
  • by P50, the inner hair cells are severely splayed
• cochlear outer hair cell degeneration (MGI Ref ID J:98396)
  • by P8, outer hair cells appear disorganized with loss of typical cell morphology
  • by P50, outer hair cells are absent, with empty patches appearing the basal portion of the cochlea
• small cochlear ganglion (MGI Ref ID J:98396)
  • described as decreased spiral ganglion

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

Tmhs^hscy/Tmhs^hscy

        involves: B6(MOR)-Tmhs^hscy/J * CAST/Ei

• behavior/neurological phenotype
• circling (MGI Ref ID J:98396)
• hearing/vestibular/ear phenotype
• absent brainstem auditory evoked potential (MGI Ref ID J:98396)
  • ABR threshold testing showed that no mutant mouse tested exhibited a response to auditory stimuli up to 110 dB sound pressure level
• circling (MGI Ref ID J:98396)
• deafness (MGI Ref ID J:98396)

View Research Applications

Research Applications

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

Tmhs^hscy related

Neurobiology Research
Vestibular and Hearing Defects

Sensorineural Research
Vestibular and Hearing Defects

Genes & Alleles

Gene & Allele Information

Allele Symbol		Tmhshscy
Allele Name		hurry-scurry
Allele Type		Spontaneous
Strain of Origin		B6.MOR-Gusba
Gene Symbol and Name		Tmhs, tetraspan transmembrane protein, hair cell stereocilia
Chromosome		17
Gene Common Name(s)		9330179O15Rik; DFNB67; LOC328789; MGC33835; RIKEN cDNA 9330179O15 gene; dJ510O8.8; hscy; hurry-scurry;
Molecular Note		This spontaneous mutation arose at The Jackson Laboratory. The mutation is a G-to-T transversion in exon 2 that results in a nonconservative amino acid change from cysteine to phenylalanine at amino acid position 161 of the encoded protein. Northern blot analysis demonstrated that a normal sized transcript was expressed from this mutant allele. Histological immunofluorescence analysis confirmed the absence of protein expression in newborn homozygous mice that is normally found in the inner and outer hair cells of the inner ear. [MGI Ref ID J:98396]

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)

Longo-Guess CM; Gagnon LH; Cook SA; Wu J; Zheng QY; Johnson KR. 2005. A missense mutation in the previously undescribed gene Tmhs underlies deafness in hurry-scurry (hscy) mice. Proc Natl Acad Sci U S A 102(22):7894-9. [PubMed: 15905332]  [MGI Ref ID J:98396]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           A1

Purchasing information

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

Pricing

Supply Details

Standard Supply

Repository-Live. A collection of over 1000 strains maintained as live colonies. Individual colonies are sized to meet current customer demand. Delivery for orders of 10 mice or less ranges on average from one to eight weeks; mice are generally shipped between four to six weeks of age with a maximum shipping age of ~nine weeks. Colony sizes do not generally support stringent age specifications for large volumes of mice; however custom orders and larger quantities of mice are easily arranged. Estimated ship dates for all orders provided within 48 hours of order placement.

Supply Notes

Usually shipped between four and eight weeks of age. This strain is included in the Mouse Mutant Resource collection. Genomic DNA is available for this strain from the Mouse DNA Resource.

Control Information

	Control
	Heterozygote from the colony
Considerations for Choosing Controls
USA, Canada and Mexico - Control Pricing Information for Genetically Engineered Mutant Strains.
International - Control Pricing Information for Genetically Engineered Mutant Strains.

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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