Beginning at approximately 10 days of age, pi/pi mice first display backing or crab-like movements and by approximately 14 days of age pirouetting is observed. This pirouetting is a spinning movement that involves swinging the head sharply to one side, either left or right, placing the inner rear foot (with the toes facing outward) close to the outer rear foot which then is used to push the mouse in a tight circling motion. This spinning persists for relatively long periods and cyclostat assessment suggests that these mutants do not develop rotational dizziness. Adult pi/pi mice can not swim on the surface of water or in a coordinated manner and are deaf at 21 to 30 days of age as determined by auditory brainstem response and a failure to respond to a variety of sounds. Although the organ of Corti develops normally, degeneration is found by the second week of life. The tectorial membrane is thickened at 12 days of age and subsequently the hair cells degenerate and the supporting cells lose form. By four weeks of age degeneration of the spiral ganglion is found. This is followed by degeneration of the saccular macula by approximately 6 weeks of age and degeneration of the stria vascularis after that. The macula of the utriculus remains unaffected. pi homozygotes differ from an array of similar labyrinth degeneration mutants in that the cristae ampullares degenerate, albeit after eight months of age. (Woolley and Dickie, 1945; Dickie and Woolley, 1946; Deol 1956; Beyer et al., 2000.)

Control Suggestions

Untyped from the colony
000664 C57BL/6J

Additional Information

Considerations for Choosing Controls

Genetics

Grxcr1^pi

Allele Symbol: Grxcr1^pi

Allele Name	pirouette
Allele Type	Spontaneous
Allele Synonym(s)
Gene Symbol and Name	Grxcr1, glutaredoxin, cysteine rich 1
Gene Synonym(s)
Strain of Origin	C3H
Chromosome	5
Molecular Note	This spontaneous mutation arose at the Jackson Laboratory in 1943. The mutation is a putative inversion with breakpoints located in intron 1 of Grxcr1 and in a region approximately 700 kb telomeric to it.

Disease/Phenotype

Disease Terms

Research Areas By Phenotype

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

Genotype: Grxcr1^pi related

Neurobiology Research
- Hearing Defects
- Vestibular Defects
Sensorineural Research
- Hearing Defects
- Vestibular Defects

Mammalian Phenotype Terms by Genotype

The following phenotype information is associated with a similar, but not exact match to this JAX^® Mice strain

Genotype: Grxcr1^pi/Grxcr1^pi
involves: C3H

hearing/vestibular/ear phenotype

vestibular saccular macula degeneration
- Normal - macula of the utriculus remains unaffected
- by 6 th weeks

cochlear hair cell degeneration
- the hair cells and the supporting cells start to degenerate by two weeks of age

stria vascularis degeneration
- after two month

abnormal vestibular system physiology
- cyclostat assessment suggests that these mutants do not develop rotational dizziness

deafness

abnormal crista ampullaris morphology
- cristae ampullares degenerate after eight months of age

organ of Corti degeneration
- although the organ of Corti develops normally, degeneration is found by the second week of life

abnormal tectorial membrane morphology
- the tectorial membrane is thickened at 12 days of age

nervous system phenotype

abnormal vestibular ganglion morphology
- a reduction of the average cell size in mice older than 200 days
- contains about the same number of cells and the range of cell sizes, however, there are much less large and more small cells

cochlear hair cell degeneration
- the hair cells and the supporting cells start to degenerate by two weeks of age

cochlear ganglion degeneration
- by four weeks of age

behavior/neurological phenotype

increased stereotypic behavior
- beginning at approximately 10 days of age mutant mice first display backing or crab-like movements
- figure eight movements
- occasional running in circles

bidirectional circling
- whirling rapidly in circles of small diameter continuously for a long time by approximately 14 days of age
- no pronounced preference for right or left turning

abnormal reflex
- no evidence of auditory response at 21 to 30 days of age to sound

head bobbing
- vertical movement of head with the nose maintained high momentarily

impaired swimming
- can not swim on the surface of water or in a coordinated manner

The following phenotype relates to a compound genotype created using this strain

Genotype: Grxcr1^pi/Grxcr1^pi
B6.C3-Grxcr1/J

cellular phenotype

absent inner hair cell kinocilia

nervous system phenotype

abnormal cochlear inner hair cell morphology
- these abnormal structure was named cytocauds
- each inner hair cell and type I vestibular hair cell contained an actin bundle that spans from the region of the cuticular plate to the basal end of the cell, then extends along with cytoplasm and membrane, towards the basement membrane

abnormal inner hair cell stereociliary bundle morphology
- appear disorganized at P30
- tip-links and kinocilia are not seen
- typical two-row organization and height gradation is absent

abnormal type I vestibular cell
- these abnormal structure was named cytocauds
- abnormal contact with the basement membrane
- each inner hair cell and type I vestibular hair cell contained an actin bundle that spans from the region of the cuticular plate to the basal end of the cell, then extends along with cytoplasm and membrane, towards the basement membrane

abnormal cochlear hair bundle tip links morphology
- inner hair cell stereicilia tip links are not seen

absent inner hair cell kinocilia

decreased inner hair cell stereocilia number
- the number of the stereocilia on each cell appear smaller

short inner hair cell stereocilia
- appear short at P30

hearing/vestibular/ear phenotype

abnormal inner hair cell stereociliary bundle morphology
- tip-links and kinocilia are not seen
- appear disorganized at P30
- typical two-row organization and height gradation is absent

deafness
- profound deafness based on ABR threshold of mutant mice at several developmental stages

abnormal cochlear inner hair cell morphology
- each inner hair cell and type I vestibular hair cell contained an actin bundle that spans from the region of the cuticular plate to the basal end of the cell, then extends along with cytoplasm and membrane, towards the basement membrane
- these abnormal structure was named cytocauds

abnormal type I vestibular cell
- abnormal contact with the basement membrane
- each inner hair cell and type I vestibular hair cell contained an actin bundle that spans from the region of the cuticular plate to the basal end of the cell, then extends along with cytoplasm and membrane, towards the basement membrane
- these abnormal structure was named cytocauds

short inner hair cell stereocilia
- appear short at P30

decreased inner hair cell stereocilia number
- the number of the stereocilia on each cell appear smaller

abnormal cochlear hair bundle tip links morphology
- inner hair cell stereicilia tip links are not seen

absent inner hair cell kinocilia

increased or absent threshold for auditory brainstem response
- after 28 days, no response at any stimulus was observed at any frequency
- responses were only obtained at 8 kHz between 14 and 28 days to a significantly increase level of stimuli of 90 dB SPL or higher

References

Additional References

Beyer LA; Odeh H; Probst FJ; Lambert EH; Dolan DF; Camper SA; Kohrman DC; Raphael Y. 2000. Hair cells in the inner ear of the pirouette and shaker 2 mutant mice. J Neurocytol 29(4):227-40PubMed: 11276175 MGI: J:94385

Additional - Grxcr1^pi related

Technical Support

CONTACT TECHNICAL SUPPORT

Genotyping Protocols
- Genotyping resources and troubleshooting
Breeding Considerations

Homozygous females will breed but often make poor mothers.
- Additional Breeding and Husbandry Support
Citation
When using the pirouette mouse strain in a publication, please cite the originating article(s) and include JAX stock #000532 in your Materials and Methods section.

Animal Health Reports

Facility Barrier Level Descriptions

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

Pricing & Availability

Cryo Recovery

Domestic
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Live Mouse

Age

Genotype

Price

weeks

Cryorecovery - Pricing

Service/Product	Description	Price
> >	Homozygous for a, Heterozygous or Wild-type for pi

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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. We do not guarantee breeding performance and therefore suggest that investigators order more than one breeding pair to avoid delays in their research.

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Licensing Information

Phone: 207-288-6470

Email: TechTran@jax.org

Also Known As:pirouette

Genetic overview

Research Applications

Base Price

Detailed Description

Control Suggestions

Additional Information

Grxcr1pi

Allele Symbol: Grxcr1pi

Disease Terms

Research Areas By Phenotype

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

Genotype: Grxcr1pi related

Mammalian Phenotype Terms by Genotype

Genotype: Grxcr1pi/Grxcr1piinvolves: C3H

hearing/vestibular/ear phenotype

nervous system phenotype

behavior/neurological phenotype

Genotype: Grxcr1pi/Grxcr1piB6.C3-Grxcr1/J

cellular phenotype

nervous system phenotype

hearing/vestibular/ear phenotype

References

Additional References

Additional - Grxcr1pi related

Genotyping Protocols

Breeding Considerations

Citation

Animal Health Reports

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

Live Mouse

Cryorecovery - Pricing

Payment Terms and Conditions

The Jackson Laboratory's Genotype Promise

Terms Of Use

Licensing Information

Grxcr1^pi

Allele Symbol: Grxcr1^pi

Genotype: Grxcr1^pi related

Genotype: Grxcr1^pi/Grxcr1^pi
involves: C3H

Genotype: Grxcr1^pi/Grxcr1^pi
B6.C3-Grxcr1/J

Additional - Grxcr1^pi related