Search Criteria: Research Area is "Sensorineural Research: Vestibular Defects"
| Stock Number |
Strain Name Strain Description |
Standard Supply |
| 006228 | B6.129S1-Nox3het-3J/GrsrJ | Repository- Live |
| Homozygotes completely lack otoconia, both in the utricle and the saccule, as early as embryonic day 14 and this persists in the adult. However, the rest of the inner ear appears morphologically normal and hearing is normal. Otoconia appear to be normal in heterozygotes. Homozygotes can be identified by a significant sideways tilting of the head. When dropped 30cm to a soft surface homozygotes fail to land on their feet. They also fail to orient and swim in water, but instead rotate underwater and require rescue. | ||
| 000305 | B6.Cg-Fbn1Tsk +/+ Pldnpa/J | Repository- Live |
| Mice homozygous for the pallid spontaneous mutation Pldnpa and nonagouti (a) have pink eyes and a light, yellow-brown coat. The Pldnpa/Pldnpa mice have a slightly lighter coat than strains that are homozygous for the pink-eyed dilution allele (Oca2p/Oca2p). Viability of homozygous mutant mice is slightly reduced. Some homozygotes have slightly abnormal behavior, with abnormal postural responses and head tilting due to the absence of otoliths in the sacculus and utriculus in many but not all mutant mice. The effect of pallid on behavior and otolith morphology appears to be a result of manganese deficiency. Homozygotes display defective mucopolysaccharide synthesis in the otolith matrix and a slower rate of transport of manganese, L-dopa, and L-tryptophane in the brain. Homozygotes have elevated basal and testosterone-induced levels of the kidney lysosomal enzymes b-glucuronidase, b-galactosi ..... For more information please see the full phenotype on the strain data sheet | ||
| 001756 | B6C3Fe a/a-Cacng2stg/J | Repository- Live |
| Mice homozygous for the spontaneous mutation stargazer (Cacng2stg) are first recognizable at 14 days of age by their smaller body size and slightly ataxic gait. Female stargazer homozygous mutant mice are fertile, but most of the males are unable to breed. The mutation is named for the abnormal head movements resembling choreiform head tossing that is characteristic of these mice. The behavioral symptoms progressively worsen with age, but both males and females will live to over one year of age. Electrocorticographical recordings of stargazer mice reveal frequent, prolonged, generalized spike-wave cortical discharges with behavioral arrest. The abnormal brain wave patterns are similar to those seen in human beings with absence epilepsy. Waggler homozygotes (Cacng2stg-wag) manifest a less severe phenotype than stargazer homozygotes, but do display an ataxic gait and occasional seizures. In general, young stargazer mice are more healthy than waggler ho ..... For more information please see the full phenotype on the strain data sheet | ||
| 004070 | CByJ.Cg-hml/GrsrJ | Repository- Live |
| Homozygotes have a faith-to-thrive phenotype such that they have a smaller body size and unbalanced gait detectable at 10 days of age. Those that survive to 2 months of age can generally live up to 18 months. The inner ear has extensive hypoplasia of the membranous labyrinth, a rudimentary tectorial membrane, and enlarged scala vestibuli and severe hearing loss or complete deafness are found at 19 days of age both by failed response to sounds and auditory brainstem response analysis. Homozygotes also fail to orient in water. | ||
| 005445 | A.B6 Tyr+-Cybanmf333/J | Cryopreserved - Ready for recovery |
| These mutants were detected through routine inspections for overt abnormal behavior; head- and body tilt can be observed at approximately 4 weeks of age (average onset 4.6 +/- 0.6 weeks; n=15). Standard pathology work-up and a whole-mount ear exam on one mutant (56 days of age) revealed no abnormalities. Male or female mutants have been produced and the colony is maintained through regular breeding.
View strain phenotype and additional information on the Neuroscience Mutagenesis Facility web page for nmf333 entry. | ||
| 000477 | B10.PA-Pldnpa H3e at/SnJ | Cryopreserved - Ready for recovery |
| This minor histocompatibility 3 (H3e) congenic strain is also carrying the closely linked pallid mutation (Pldnpa) as well as the black and tan allele of nonagouti also residing on Chromosome 2. Histocompatibility 3 is one of several loci isolated and identified by Dr. George Snell and co-workers. Histocompatibility gene differences were identified by generating congenic resistant strains and testing for resistance to transplantable tumors. Homozygous pallid mice have pink eyes, a diluted coat color and their viability is slightly reduced. Some homozygotes have slightly abnormal behavior, with abnormal postural responses and head tilting due to the absence of otoliths in the sacculus and utriculus in many but not all mutant mice. The effect of pallid on behavior and otolith morphology appears to be a result of manganese deficiency. Homozygotes display defective mucopolysaccharide synthesis in the otolith matrix and a slower rate of transport of manga ..... For more information please see the full phenotype on the strain data sheet | ||
| 003483 | B6 x B10.D1-H2q/SgJ-Nox3het-2J/J | Cryopreserved - Ready for recovery |
| 004835 | B6 x B6JCu.Cg-wl/J | Cryopreserved - Ready for recovery |
| Homozygous wabbler-lethal mice are first recognizable at 12 days of age and usually die at about 4 weeks. They have an abnormal wobbly gait and a pronounced tremor when walking. | ||
| 000061 | B6 x STOCK Nox3het/J | Cryopreserved - Ready for recovery |
| Head tilt (Nox3het) is an autosomal recessive mutation that can cause abnormal circling behavior and hyperactivity in affected mice. Homozygotes also exhibit a subtle head tilt. Together, the abnormal behavioral phenotype is consistent with that of a vestibular disorder. Evoked auditory brainstem response profiles are normal indicating that the mutants are not deaf. Nox3het/Nox3het mutants are unable to sense orientation under water and therefore, cannot swim properly. If held by the tail, Nox3het/Nox3het mice retract, rather than extend, their limbs; they also flex ventrally, instead of dorsally as wild type mice do. When lowered quickly by the tail, Nox3het/Nox3het mice fail to extend their forelimbs in a normal manner and have difficulty righting themselves if dropped vertically from a short distance. Morphological assessment of the inner ear of homozygotes r ..... For more information please see the full phenotype on the strain data sheet | ||
| 000578 | B6 x STOCK Tyrc-ch Bmp5se +/+ Myo6sv/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the Snell's waltzer spontaneous mutation (Myo6sv) show to a marked degree the typical circling, head-tossing, deafness, and hyperactivity of other mutant mice of this type. Homozygous mutant mice are recognizable by the age of 1 week. The abnormalities of the inner ear consist of degeneration of the entire neuroepithelium comprising the organ of Corti, the saccular and utricular maculae, and the cristae of all three semicircular canals. Although viability of homozygotes is nearly normal, breeding ability is reduced and males are more reliable breeders than females.
Specific cytoskeletal components are critical for specific cellular structures. The microvilli of intestinal brush border cells in Myo6sv homozygotes are shorter than normal. While myosin 6 is not critical for the development of hair cell stereocilia, it is essential for their maintenance. At birth the stereocilia appear nearly normal with only occasional stereocil ..... | ||
| 008834 | B6(Cg)-Tmiesr-2J/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the spinner 2 Jackson mutation display head tilting and circling and are deaf. | ||
| 004640 | B6(MOR)-Lhfpl5hscy/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the Lhfpl5hscy 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). | ||
| 002552 | B6(V)-Cdh23v-2J/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the waltzer 2J spontaneous mutation (Cdh23v-2J) show the circling, head-tossing, deafness, and hyperactivity typical of the circling mutants. Homozygous mutant mice are very similar to the other waltzer mutants (Cdh23v and Cdh23v-J). Most homozygotes are deaf from birth. Abnormalities of the inner ear include degeneration of the organ of Corti, spiral ganglion, stria vascularis, and saccular macula. Double heterozygotes with shaker-1 (v/+ Myo7ash1/+) are deaf beginning at 3 to 6 months. Double heterozygotes have changes similar to those of the homozygotes in the organ of Corti, stria vascularis, and spiral ganglion, but less severe and with much later onset. Viability and breeding ability are somewhat reduced. | ||
| 004276 | B6.129-Figntm1Frk/Frk | Cryopreserved - Ready for recovery |
| 006853 | B6.129-Ush1ctm1Xzl/Kjn | Cryopreserved - Ready for recovery |
| Mice homozygous for the Ush1ctm1Xzl mutation have circling, head-tossing, and hyperactive behaviors typical of vestibular mutants. Auditory-evoked brainstem assessment shows that homozygotes are completely deaf and scanning electron microscopy shows that the hair cell stereocilia become progressively disorganized with age, with the outer hair cells more affected than the inner hair cells. Although histology at 12 months of age does not reveal any abnormalities, there is a decline in electrotreinogram readings for both rods and cones by 11 months of age. Because the first four exons were replaced by the beta galactosidase reporter cassette, endogenous expression of this gene in phenotypically normal mice can be traced by assessing lacZ expression in heterozygotes. | ||
| 000783 | B6.A-Ush1gjs/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the Ush1gjs allele are deaf, circle, and toss their heads from side to side. They are viable and fertile but due to their circling gait females may trample their pups and thus should not be used for breeding. No auditory brain response was detected from homozygotes at 14, 21, and 30 days of age. At 10-14 days of age the stereocilia of the outer and macular hair cells are disarrayed or missing although no abnormal phenotype is evident in inner hair cells, or in the gross anatomy of the cochlea or vestibule. The steriocilia degeneration in outer hair cells continues but the inner hair cells remain normal at 30-47 days of age. (Dickie and Deol, 1967; Kitamura et al., 1992.) | ||
| 004202 | B6.C3 Pde6brd1 Hps4le/+ +-Lmx1adr-8J/J | Cryopreserved - Ready for recovery |
| 000532 | B6.C3-Grxcr1pi/J | Cryopreserved - Ready for recovery |
| 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 degenera ..... For more information please see the full phenotype on the strain data sheet | ||
| 002624 | B6.C3-Lmx1adr-6J/J | Cryopreserved - Ready for recovery |
| 004275 | B6.Cg-Fignfi/Frk | Cryopreserved - Ready for recovery |
| 007787 | B6.Cg-Grxcr1pi-4J/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the pirouette 4 Jackson mutation display bi-directional circling and are completely deaf as early as 5 weeks of age. Consistent with a vestibular defect, they fail to swim at the surface of water, but instead roll underwater and sink before rescue. | ||
| 000071 | B6.Cg-Mcoln3Va/J | Cryopreserved - Ready for recovery |
| Mice heterozygous for the varitint-waddler spontaneous mutation (Mcoln3Va) are deaf and show circling behavior, head-tossing, and hyperactivity. Heterozygotes circle somewhat less than some of the other circling mutants. Their coats are variegated with patches of normal-colored, diluted, and white fur. Homozygotes show more intense behavioral abnormalities than heterozygotes, and their coats are white, except for small patches of unaltered color near the ears and base of the tail. The pathological changes in heterozygotes include degeneration of the organ of Corti, stria vascularis, spiral ganglion, saccular macula, cristae ampullares, and vestibular ganglion. In homozygotes the degenerative changes are more severe and also include the utricular macula. Viability of heterozygotes is nearly normal, but fertility is reduced. Mortality is very high in homozygotes, and very few of the survivors are fertile. Compound heterozygotes for the two alleles (Mcoln3Va-J> ..... For more information please see the full phenotype on the strain data sheet | ||
| 006124 | B6.Cg-Myo6sv-2J/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the Snell?s waltzer 2 Jackson mutation display circling and head tossing behavior and are deaf. They are unable to orient in water to swim. | ||
| 003184 | B6.Cg-Myo7ash1-8J/J | Cryopreserved - Ready for recovery |
| Myo7ash1-8J homozygous mice circle and bob their heads; heterozygotes are behaviorally normal. Homozygotes of both sexes are viable and fertile (Samples 2000). The hearing of Myo7ash1-8J mutants has not been examined. However, other Myo7a mutations, whose behavioral phenotypes are virtually identical to that of Myo7ash1-J, are associated with deafness. Mutations in the human ortholog of Myo7a, MYO7A, have been characterized in pedigrees of patients having Usher syndrome type 1 (USH1), an autosomal recessive disorder characterized by profound deafness, vestibular dysfunction and progressive retinitis pigmentosa (Weil et al., 1995; Weston et al., 1996; Adato et al., 1997; Levy et al., 1997; Liu et al., 1998; Espinos et al., 1998; Cuevas et al., 1998, 1999; Janecke et al., 1999). MYO7A mutations have also been identified in patients with autosomal dominant non-sy ..... | ||
| 001104 | B6.Cg-Otop1tlt/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the Otop1tlt mutation are viable and fertile. They can be identified outwardly by a tilt in the head posture, which is reported to be detectable in 50-75% of homozygotes. Auditory brainstem responses at four months of age are normal. Although these mice are not deaf and do not show degeneration of the cochlea or vestibular organs, most completely lack otoconia in both the utricle and saccule and thus have problems with spatial orientation. A few giant otoconia have been observed infrequently in a small minority of these mutants, but generally there is agenesis of otoconia. The macular epithelium and otolithic membrane are intact despite the lack of otoconia. Homozygotes are unable to orient themselves in water to find the surface and thus are categorized as non-swimming since they would drown without rescue. Approximately 2% of these homozygotes are able to find the water surface despite somersaults, backflips, or other abnormal motions indic ..... For more information please see the full phenotype on the strain data sheet | ||
| 000024 | B6.Cg-Pldnpa/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the pallid spontaneous mutation pa and nonagouti (a) are pink-eyed and have a light, yellow-brown coat. The coat is a little lighter than that of pink-eyed dilution homozygotes (p/p). Viability of homozygote mutant mice is slightly reduced. Some homoygotes have slightly abnormal behavior, with abnormal postural responses and head tilting due to the absence of otoliths in the sacculus and utriculus in many but not all mutant mice. The effect of pallid on behavior and otolith morphology appears to be a result of manganese deficiency. Homozygotes display defective mucopolysaccharide synthesis in the otolith matrix and a slower rate of transport of manganese, L-dopa, and L-tryptophane in the brain. Homozygotes have elevated basal and testosterone-induced levels of the kidney lysosomal enzymes b-glucuronidase, b-galactosidase, and a-mannosidase, accompanied by lowered enzyme excretion in the urine. Pallid mice have a deficiency in serum a1-antitrypsi ..... For more information please see the full phenotype on the strain data sheet | ||
| 000571 | B6.Cg-Whrnwi Tyrp1b/+ +/J | Cryopreserved - Ready for recovery |
| At 9 to 10 days of age Whrnwi 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 ..... | ||
| 007757 | B6.Cg-hml/J | Cryopreserved - Ready for recovery |
| Homozygotes have a faith-to-thrive phenotype such that they have a smaller body size and unbalanced gait detectable at 10 days of age. Those that survive to 2 months of age can generally live up to 18 months. The inner ear has extensive hypoplasia of the membranous labyrinth, a rudimentary tectorial membrane, and enlarged scala vestibuli and severe hearing loss or complete deafness are found at 19 days of age both by failed response to sounds and auditory brainstem response analysis. Homozygotes also fail to orient in water. | ||
| 004620 | B6.L-Whll/J | Cryopreserved - Ready for recovery |
| Whll/+ mice have a moderate circling phenotype accompanied by a mild hearing impairment. | ||
| 004768 | B6;129S4-Ush1cdfcr-2J/J | Cryopreserved - Ready for recovery |
| Mice homozygous for either Ush1cdfcr or Ush1cdfcr-2J display head tossing and hyperactive circling. They are unable to orient themselves in water to swim at the surface. Despite these behavioral traits, these mice are able to breed and have normal life spans. Adult homozygotes have no auditory-evoked brainstem response. This complete deafness was found in an Ush1cdfcr homozygote as early as 21 days of age. Scanning electron micrographs of the organ of Corti at 3 weeks of age reveal disorganized and splayed stereocilia of outer hair cells and, less severely, of inner hair cells along with degeneration of hair cells that yields a disorganized hair cell pattern. While progressive loss of hair cells and a secondary loss of spiral ganglion cells are found and are severe by 8 months of age, no gross morphological abnormalities were discerned in whole mounts of inner ears. At 9 months of age a slight peripheral retinal degeneration i ..... For more information please see the full phenotype on the strain data sheet | ||
| 008645 | B6;129S7-Pou4f3tm1Xia/J | Cryopreserved - Ready for recovery |
| These mice carry a targeted mutation of the POU domain, class 4, transcription factor 3 gene. Homozygotes are deaf, have impaired balance, and display a robust circling behavior due to defects of the inner ear. Auditory and vestibular hair cells are lost during late embryonic and early postnatal periods. A secondary loss of spiral and vestibular ganglion neurons is also seen. This strain may be useful in studies of auditory and vestibular system development. | ||
| 003892 | B6;BKS-Atp2b2dfw-3J/J | Cryopreserved - Ready for recovery |
| 006033 | B6;CByJ-nmf445/J | Cryopreserved - Ready for recovery |
| View strain phenotype and additional information on the Neuroscience Mutagenesis Facility web page for nmf445 entry. | ||
| 000126 | B6By.Cg-Sd Mcoln3Va-J Krt25Re/J | Cryopreserved - Ready for recovery |
| Mice heterozygous for the varitint-waddler Jackson spontaneous mutation (Mcoln3Va-J) are more pigmented than the original varitint waddler mice (Mcoln3 Va) and behave normally although they are deaf. They have slightly diluted coat color, a large irregular belly spot, and white feet and tail tip. Homozygous mutant mice have extensive white spotting interspersed with patches of diluted color. They are deaf but behave normally and are fertile. Compound heterozygotes for the two alleles (Mcoln3Va-J/Mcoln3Va) are similar to Mcoln3Va-J/Mcoln3Va-J mice but are smaller with more white spotting and abnormal behavior. They are deaf and circle vigorously. Viability and fertility of Mcoln3Va-J/Mcoln3Va mice are considerably reduced. This strain is also carrying two other mutations, rex (Krt25Re) and Danforth's short tail (Sd). | ||
| 000636 | B6C3Fe a/a-Lmx1adr-J/J | Cryopreserved - Ready for recovery |
| Lmx1a encodes a LIM homeodomain (LIM-hd) protein that is expressed in the roof plate along the neuraxis during CNS development. The Lmx1adr-J mutation replaces a conserved cysteine in the LIM1 domain of the protein with a tyrosine (Millonig et al. 2000). Mice homozygous for Lmx1adr-Jare identifiable at birth by a thin filament extending from the end of the tail, which drops off within a few days, and a short or blunt tail. They may also have a head bleb. When fur develops, there may be a white belly spot. Lmx1adr-J/Lmx1adr-J mice are ataxic and hyperactive and have difficulty righting themselves, and they do not reproduce (Sweet and Wahlsten 1983). They also exhibit circling behavior, are deaf, and have inner ear and vertebral malformations (Manzanares et al. 2000).
Lmx1adr-J homozygotes exhibit defects of three classes of anatomic structures: the hindbrain roof plate, neural crest derived ..... | ||
| 000296 | B6C3Fe-a/a Hoxa13Hd Mcoln3Va-J/J | Cryopreserved - Ready for recovery |
| Mice heterozygous for the varitint-waddler Jackson spontaneous mutation (Mcoln3Va-J) are more pigmented than the original varitint waddler mice (Mcoln3Va) and behave normally although they are deaf. They have slightly diluted coat color, a large irregular belly spot, and white feet and tail tip. Homozygous mutant mice have extensive white spotting interspersed with patches of diluted color. They are deaf but behave normally and are fertile. Compound heterozygotes of the two alleles (Mcoln3Va-J/Mcoln3Va) are similar to Mcoln3Va-J/Mcoln3Va-J mice but are smaller with more white spotting and abnormal behavior. They are deaf and circle vigorously. Viability and fertility of Mcoln3Va-J/Mcoln3Va mice are considerably reduced. This strain is also carrying the semidominant hypodactyly spontaneous mutation (Hoxa13Hd). Heterozygous hyp ..... For more information please see the full phenotype on the strain data sheet | ||
| 002557 | B6Ei.GL-Nox3het/J | Cryopreserved - Ready for recovery |
| Head tilt (het) is an autosomal recessive mutation that can cause abnormal circling behavior and hyperactivity in affected mice. Homozygotes also exhibit a subtle head tilt. Together, the abnormal behavioral phenotype is consistent with that of a vestibular disorder. Evoked auditory brainstem response profiles are normal indicating that the mutants are not deaf. het/het mutants are unable to sense orientation under water and therefore, cannot swim properly. If held by the tail, het/het mice retract, rather than extend, their limbs; they also flex ventrally, instead of dorsally as wild type mice do. When lowered quickly by the tail, het/het mice fail to extend their forelimbs in a normal manner and have difficulty righting themselves if dropped vertically from a short distance. Morphological assessment of the inner ear of homozygotes reveals an abnormal appearance of the saccule and utricle owing to a complete absence of otoliths. Otoliths are tiny cal ..... For more information please see the full phenotype on the strain data sheet | ||
| 004771 | BALB/cBy-Ush1cdfcr/J | Cryopreserved - Ready for recovery |
| Mice homozygous for either Ush1cdfcr or Ush1cdfcr-2J display head tossing and hyperactive circling. They are unable to orient themselves in water to swim at the surface. Despite these behavioral traits, these mice are able to breed and have normal life spans. Adult homozygotes have no auditory-evoked brainstem response. This complete deafness was found in an Ush1cdfcr homozygote as early as 21 days of age. Scanning electron micrographs of the organ of Corti at 3 weeks of age reveal disorganized and splayed stereocilia of outer hair cells and, less severely, of inner hair cells along with degeneration of hair cells that yields a disorganized hair cell pattern. While progressive loss of hair cells and a secondary loss of spiral ganglion cells are found and are severe by 8 months of age, no gross morphological abnormalities were discerned in whole mounts of inner ears. At 9 months of age a slight peripheral retinal degeneration is fou ..... For more information please see the full phenotype on the strain data sheet | ||
| 006274 | BALB/cByJ-Dfb/J | Cryopreserved - Ready for recovery |
| Mice heterozygous for the deaf ballerina mutation display circling and head tossing behavior, are deaf, and lack the corpus callosum. Heterozygous females do not breed. | ||
| 003929 | BXA4/Pgn-Tmiesr-J/J | Cryopreserved - Ready for recovery |
| 006816 | BXA7/Pgn-Slc26a4pdsm/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the pdsm mutation display head bobbing, circling, and occasional head tilt, evident by 3 weeks of age. The inner ear defects include diminished or absent otoconia, hair cells and spiral ganglion cells, malformed tectorial membrane, a reduction in the number of cochlear turns, degeneration of the organ of Corti, and displacement of Reissner's membrane resulting in enlarged scala media. Serum chemistry and histology failed to detect any signs of hypothyroidism. | ||
| 001276 | C3H/HeJ-Atp2b2dfw/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the deaf waddler spontaneous mutation (Atp2b2dfw) are deaf, walk with a hesitant, wobbly gait, and bob their heads. Both sexes are fertile. | ||
| 004406 | C3HeB/FeJ-Pou3f4del-J/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the Pou3f4del-J display head shaking and circling behavior by three weeks of age. Homozygous females and males hemizygous for this X-linked mutation have profound deafness, although heterozygous females do not. They have cochlear hypoplasia, a reduced number of cochlear turns, failure to fully form the bony structure of the modiolus, and detachment of the stria vascularis. | ||
| 004764 | C57BL/6J-Cdh23v-8J/J | Cryopreserved - Ready for recovery |
| The mutants were initially detected based on abnormal pre-pulse-inhibition test results. They were subsequently observed to exhibit a slight head tilt and circling behavior (onset at approximately 4 weeks of age), and the mutant line was established based on the overt phenotype. Testing of auditory brainstem responses revealed the mutants (n=4) to be deaf. The vestibular abnormality of nmf112 was mapped as a recessive phenotype in 19 affected and 20 unaffected (C57BL/6J-nmf112 x BALB/cByJ) F2 intercross mice, and the best linkage was to D10Mit138 (LOD = 7.0) and D10Mit15 (LOD = 6.1). Because of the map position of this mutation and its phenotypic similarity to the waltzer mutations, a series of complementation tests was performed to determine if allelic relationships exist between NMF112, NMF181, NMF252 and Cdh23. The results showed NMF112 to be an allele of NMF181 (one mating produced 2 mutants in a total of 7 progeny), of NMF252 (one mating produced 2 mutants in a total of 5 p ..... For more information please see the full phenotype on the strain data sheet | ||
| 004819 | C57BL/6J-Cdh23v-9J/J | Cryopreserved - Ready for recovery |
| The mutants exhibit circling and head tossing behavior by weaning age (at 3-4 weeks), and three animals tested at four weeks of age for Auditory brain stem response were deaf. Mutants of either gender have been produced and the colony is maintained through regular breeding. Serial sections of the ears of two mutants (24 and 29 days of age) and additional whole ear histology on one mutant (33 days of age) revealed no abnormalities. Standard pathology work-up on all three animals showed no other abnormalities. Because of the map position of this mutation, and its phenotypic similarity to the waltzer mutations, a series of complementation tests has been performed to determine if allelic relationships exist between NMF181, NMF112, NMF252 and Cdh23. The results showed NMF181 to be an allele of NMF112(one mating produced 2 mutants in a total of 7 progeny), of NMF252(one mating produced 2 mutants in a total of 8 progeny) and therefore of Cdh23, since NMF252 has been identified a ..... For more information please see the full phenotype on the strain data sheet | ||
| 004820 | C57BL/6J-Kcne12J/J | Cryopreserved - Ready for recovery |
| The mutants show circling and head tossing behavior, and a slight head tilt, observable at 4 weeks of age (+/-1 week; n=18). Two mutants tested for Auditory brainstem responses were found to be deaf. Serial sections of the ears of one mutant (32 days of age) showed a thickened tectorial membrane with mineralization and adhesion to Reissner's membrane; whole ear exam of one other mutant (30 days of age) showed abnormally shaped maculae. Standard pathology work-up showed no other abnormalities.
Because of the map position of this mutation, and its phenotypic similarity to Kcne1 mutations (potassium voltage-gated channel, Isk-related subfamily, member 1, see also Vetter et al., 1996), complementation tests with Kcne1pkr (Kcne1 punk rocker Letts et al., 2000) have been performed to determine if NMF190 represents an allele of Kcne1. Two litters of heterozygote matings between NMF190 and Kcne1pkr yielded 2 mutants in a total of 12 pro ..... | ||
| 005749 | C57BL/6J-Myo6sv-3J/J | Cryopreserved - Ready for recovery |
| 005468 | C57BL/6J-Myo7ash1-11J/J | Cryopreserved - Ready for recovery |
| The mutants were identified through head tilt, body leaning and bi-directional circling behavior at wean (average onset at 3.5 +/- 0.6 weeks of age; n=31); they also show head tossing behavior. ABR testing showed the mutants to be deaf at all frequencies (8, 16, 32KHz) tested. Because of phenotypic and genotypic similarities to mutants of Myo7a, complementation tests were performed with B6.Cg-Myo7ash1-8J/J (JR#3184); the results of two heterozygous by homozygous matings (5 affected in a total of 24 progeny) suggest that nmf371 represents an allele of Myo7a. Serial sections of the ears of two mutants (160 days of age) revealed a virtual absence of organ of corti,spiral ganglion cells, hair cells or normal otoconia; whole ear exam also showed defective otoconia. Standard pathology work-up revealed no further abnormalities.
View strain phenotype and additional information on the Neuroscience Muta ..... | ||
| 005014 | C57BL/6J-Nox3het-4J/J | Cryopreserved - Ready for recovery |
| These mutants were detected through routine inspections for overt abnormal behavior and the onset of head- and body tilt has been determined to be at 4 weeks of age (+/- 0.3 weeks); occasional circling behavior has also been observed. Results of Auditory brainstem recordings (n=2) were normal. Complementation analysis, i.e. matings between heterozygous nmf250 (female) and homozygous Nox3 mice (male, JR#002557, B6Ei.GL-Nox3het/J) resulted in 5 mutants in a total of 7 progeny, demonstrating that nmf250 represents an allele of Nox3. Standard pathology work-up on two mutants (43 or 112 days of age) revealed no abnormalities. Serial sections of the ears of the older mouse showed a lack of otoconia in the ear, which was confirmed by examination of ear whole mounts of two other mutants. No other abnormalities were observed. Male or female mutants have been produced and the colony can usually be maintained through homozygous matings.
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| 003484 | C57BL/6J-Pou4f3ddl/J | Cryopreserved - Ready for recovery |
| 000563 | C57BL/6J-Sobpjc/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the Jackson circler spontaneous mutation (Sobpjc) show erratic circling behavior which becomes more pronounced with age. Homozygous mutant mice are very active, bobbing up and down and flexing head and trunk ventrally towards the tail and mice can swim. Mice can be identified at weaning by a characteristic flexing behavior when suspended by tail. | ||
| 000543 | C57BL/6J-Tmiesr/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the spinner spontaneous (Tmiesr) show the typical head tossing, circling, deafness, and hyperactivity of the shaker and waltzer mutants. Abnormal behavior in homozygous mutant mice can be recognized as early as 7 days. Inner ear abnormalities consist of degeneration of the organ of Corti and spiral ganglion, reduction in size of the stria vascularis of the cochlea, and degeneration of the saccular macula. Both sexes are fertile, but females are not good mothers. | ||
| 004818 | C57BL/6J-nmf172/J | Cryopreserved - Ready for recovery |
| The mutants exhibit (bi-directional) circling and head tossing behavior by 4 weeks of age, and of 3 mutants tested for auditory brainstem responses (at 6 weeks of age) 2 were found to be deaf and 1 hearing impaired. Standard pathology work-up on two mutants (95 or 173 days of age), including serial sections of the ears of one animal (95 days of age) showed no abnormalities. Whole ear exam performed on a third mutant (47 days of age) also showed no abnormalities.
View strain phenotype and additional information on the Neuroscience Mutagenesis Facility web page for nmf172 entry. | ||
| 004831 | C57BL/6J-nmf219/J | Cryopreserved - Ready for recovery |
| The mutants exhibit circling and head tossing behavior by weaning age (average date of onset 3.7 weeks of age +/- 0.66; n=20), and two animals tested for Auditory brainstem response at 6 weeks of age were found to be deaf. Standard pathology work-up on four mutants (29, 36 or 111 days of age) revealed abnormal development of the ears of three mutants. Serial sections of the ears of a 29 and a 111 day old mutant showed a lack of organization, no properly formed hair cells were observed in the older mouse; the semicircular canals also appeared very small. However, whole ear histology performed on an additional mutant (29 days of age) revealed no abnormalities. In the 36 day old mutant, macrophages were present in spinal and trigeminal nerve roots, as well as in the white matter of the CNS. No other abnormalities were observed. Male or female mutants have been produced and a colony can be maintained through heterozygote or mutant breeding (though heterozygotes breed better than mutants).
..... For more information please see the full phenotype on the strain data sheet | ||
| 006169 | CBA.Cg-Tmc1dn/AjgJ | Cryopreserved - Ready for recovery |
| Homozygotes for this spontaneous mutation exhibit degeneration in the spiral ganglion neurons as well as the organ of Corti and the saccular macula. Mice are deaf throughout life. This strain may be used to research inherited deafness. | ||
| 002894 | CByJ.A-Atp2b2dfw-2J/J | Cryopreserved - Ready for recovery |
| The deaf waddler 2J allele (Atp2b2dfw-2J) arose spontaneously in a congenic substrain of BALB/cByJ mice, CByJ.A-fsn/J. Homozygous mutant mice are viable, but neither sex will breed. They are recognizable by 12-14 days of age by their hesitant, wobbly gait and head bobbing behavior. Homozygotes also develop a slight body tremor and become increasingly lethargic. Homozygous mutant mice are profoundly deaf, exhibiting no discernible auditory brainstem responses (ABR) to stimuli up to 100 dB. Heterozygous Atp2b2dfw-2J mutant mice also show age-dependent changes in ABR and progressive hearing loss as a result of a modifier allele (ahl) present in BALB/cByJ mice. | ||
| 005016 | CByJ;B6-Cdh23v-10J/J | Cryopreserved - Ready for recovery |
| The mutants exhibit circling behavior and a slight head tilt and tossing behavior by weaning age; two mice tested for Auditory brain stem response (at 6 weeks of age) were found to be deaf. Because of the map position of this mutation, and its phenotypic similarity to the waltzer mutations, a complementation test has been performed to determine if NMF252 represents an allele of Cdh23. One mating between NMF252 and Cdh23v-2J mice produced 2 mutants in a total of 6 progeny, suggesting that NMF252 represents a new allele of Cdh23. Therefore, NMF252 mutants might be useful for studying neurobiological mechanisms related to Usher syndrome and human deafness. Standard pathology on two mutants (49 days of age) revealed no abnormalities; whole ear exam and serial sections of the ears also showed no abnormalities. Male or female mutants have been produced, and a colony can be maintained through homozygous matings.
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| 004641 | DBA/2J-Grxcr1pi-2J/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the pirouette 2 Jackson mutation display bi-directional circling and head bobbing and are completely deaf by ABR assessment. Consistent with a vestibular defect, they fail to swim at the surface of water, but instead roll underwater and sink before rescue. | ||
| 000619 | FS/EiJ | Cryopreserved - Ready for recovery |
| The FS/Ei strain was originally used as a linkage testing stock for gene mapping. It is homozygous for several visible recessive mutations including brown (Tyrp1b), pink-eyed dilution (Oca2p), chinchilla (Tyrc-ch), frizzy (fr), and the neurological mutation shaker 1 (Myo7ash1). It is also homozygous for a couple allelic variants that can be easily typed (Mod2b and Hbbd). Mice homozygous for the shaker 1 show circling, head-tossing, deafness, and hyperactivity characteristic of this type of mutant mice. Viability is normal, and breeding ability is high for a circling mutant. Homozygous mutant mice are most often deaf and swim well on the surface of water up to 4 weeks of age and more but lose the ability later. The degenerative changes of the labyrinth may occur a little later than in some of the other waltzing mutants. By light microscopy, the changes are seen to consist ..... For more information please see the full phenotype on the strain data sheet | ||
| 002757 | MK;B6-Slc12a2sy-ns/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the shaker with no syndactylism spontaneous mutation (Slc12a2sy-ns) is allelic with the original radiaton-induced shaker-with-syndactylism mutation (sy). Homozygous mutant mice exhibit deafness and balance defects but have a normal foot morphology. Two other spontaneous alleles of sy, fused phalanges and fused phalanges 2 Jackson, do not affect hearing or behavior. Tests for allelism among mice with four different mutant alleles at the shaker-with-syndactylism locus (sy, syfp, syfp-2J, and Slc12a2sy-ns) indicate that sy is a deletion including at least two genes associated with distinct phenotypes. Mice homozygous for sy have syndactylous feet and other skeletal malformations, are deaf, and exhibit abnormal behavior characteristic of vestibular dysfunction. Complementation test results indicate that sy, syfp and syfp-2J> ..... For more information please see the full phenotype on the strain data sheet | ||
| 000268 | RSV/LeJ | Cryopreserved - Ready for recovery |
| Mice heterozygous for the varitint-waddler spontaneous mutation (Mcoln3Va) are deaf and show circling behavior, head-tossing, and hyperactivity. Heterozygotes circle somewhat less than some of the other circling mutants. Their coats are variegated with patches of normal-colored, diluted, and white fur. Homozygotes show more intense behavioral abnormalities than heterozygotes, and their coats are white, except for small patches of unaltered color near the ears and base of the tail. The pathological changes in heterozygotes include degeneration of the organ of Corti, stria vascularis, spiral ganglion, saccular macula, cristae ampullares, and vestibular ganglion. In homozygotes the degenerative changes are more severe and also include the utricular macula. Viability of heterozygotes is nearly normal, but fertility is reduced. Mortality is very high in homozygotes, and very few of the survivors are fertile. Compound heterozygotes for the two alleles (Mcoln3Va-J> ..... For more information please see the full phenotype on the strain data sheet | ||
| 000271 | SH1/LeJ | Cryopreserved - Ready for recovery |
| Mice homozygous for the shaker 1 spontaneous mutation (Myo7ash1) show circling, head-tossing, deafness, and hyperactivity characteristic of this type of mutant mice. Viability is normal, and breeding ability is high for a circling mutant. Homozygous mutant mice are most often deaf and swim well on the surface of water up to 4 weeks of age and more but lose the ability later. The degenerative changes of the labyrinth may occur a little later than in some of the other waltzing mutants. By light microscopy, the changes are seen to consist of degeneration of the organ of Corti, the spiral ganglion, and the stria vascularis in the cochlea, and of the saccular macula and the vestibular ganglion in the vestibular labyrinth. | ||
| 003961 | SJL.Cg Thy1a-Noxo1hslt/J | Cryopreserved - Ready for recovery |
| 000109 | STOCK Myo15sh2/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the shaker 2 spontaneous mutation (Myo15sh2) are viable and fertile, showing only a slight reduction in both compared to wildtype mice. Homozygous mutant mice display a phenotype very similar to the behavior and pathology to shaker-1 (Myo7ash1) with the exception that the abnormalities are observed a little earlier in shaker 2 mice. Homozygotes appear to be deaf from the beginning, and the saccular macula is abnormal at birth. The stria vascularis appears normal by light microscopy at 2 weeks, but begins to show degenerative changes shortly thereafter. Many of the cells contain electron-dense inclusions filled with a fine granular material. At 2.5 months, the type I hair cells of the cristae ampullares and maculae utriculi contain rod-shaped inclusion bodies composed of actin filaments. The visible phenotype of the shaker-2 Jackson (Myo15sh2-J, Stock No. 002048<> ..... For more information please see the full phenotype on the strain data sheet | ||
| 002919 | STOCK Myo7ash1-7J/J | Cryopreserved - Ready for recovery |
| Myo7ash1-7J homozygotes exhibit circling behavior, a slight head tilt and side-to-side head bob. They develop progressive hearing loss, becoming totally deaf by 60 days of age. Heterozygotes are normal. (Samples et al., 2000). Mutations of the human ortholog of Myo7a, MYO7A, have been characterized in pedigrees of patients having Usher syndrome type 1 (USH1), an autosomal recessive disorder characterized by profound deafness, vestibular dysfunction and progressive retinitis pigmentosa (Weil et al., 1995; Weston et al., 1996; Adato et al., 1997; Levy et al., 1997; Liu et al., 1998; Espinos et al., 1998; Cuevas et al., 1998, 1999; Janecke et al., 1999). MYO7A mutations have also been identified in patients with autosomal dominant non-syndromic deafness (Liu et al., 1997) and autosomal recessive isolated deafness (Liu et al., 1997; Weil et al., 1997). | ||
| 006104 | STOCK Ush1cdfcr-3J/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the deaf circler 3 Jackson mutation display head bobbing and rapid circling and are deaf. This strain is maintained segregating for coat color alleles resulting in agouti and albino mice. | ||
| 000312 | STOCK stb + a/+ Fignfi a/J | Cryopreserved - Ready for recovery |
| Mice homozygous for the fidget spontaneous mutation (Fignfi) toss their heads from side to side and tend to run in circles. Homozygous fidgit mice are hypersensitive to sound in early life, but later lose hearing ability. Corneal lesions are common, and hind foot polydactylism occurs. The bony labyrinth is quite defective. Other abnormalities including small eyes, absence of lachrymal glands, dislocation of the hip, and displaced parafloccular lobes of the cerebellum. Viability of homozygotes is usually less than normal and fertility is poor. The STOCK is also carrying the stubby spontaneous mutation (stb). Homozygous stubby mice are recognizable at 4 or 5 days by a domed head and thick short tail. Adult stubby mice have shorter heads, bodies, and legs compared to wildtype mice. Most females are fertile but males do not breed. Male mice do produce normal numbers of motile sperm. Cartilage histology is within normal limits, but chondrogenesis stops earlier than ..... For more information please see the full phenotype on the strain data sheet | ||
| 000275 | V/LeJ | Cryopreserved - Ready for recovery |
| This strain was originally used as a linkage stock for gene mapping. It is homozygous for several visible recessive mutations all located on different chromosomes including nonagouti (a), fuzzy (fz), leaden (Mlphln), and piebald (Ednrbs) and is segregating for the neurological mutation, waltzer (Cdh23v). Homozygous waltzer mice show the circling, head-tossing, deafness, and hyperactivity typical of the circling mutants. Abnormalities of the inner ear include degeneration of the organ of Corti, spiral ganglion, stria vascularis, and saccular macula. Homozygous piebald mice show irregular white spotting, the amount of which is greatly influenced by minor modifying genes. Homozygous piebald mice have dark eyes. The white areas of the coat are completely lacking in melanocytes and there is a reduction in the number of melanocytes in the choroid layer of the eye. | ||
| 000147 | WLHR/LeJ | Cryopreserved - Ready for recovery |
| WLHR/Le is a balanced stock with wabbler-lethal (wl) and hairless (hr) spontaneous mutations maintained in repulsion on Chromosome 14. Homozygous wabbler-lethal mutant mice are first recognizable at 12 days of age and usually die at about four weeks. They have an abnormal wobbly gait and a pronounced tremor when walking. In an extensive study of behavioral development of this mutant, homozygous wabbler-lethal mice were shown to be deficient in nearly all behaviors tested. Histological examination showed myelin degeneration widely distributed throughout the CNS, particularly in the vestibulocerebellar and spinocerebellar systems. Electron microscopy showed widespread axonal (Wallerian) degeneration in the medulla with secondary myelin dissolution. Similar abnormalities were present to a lesser extent in the basal ganglia, spinal cord, and cerebellum and in the optic nerve. Homozygous hairless mutant mice have a higher incidence and earlier onset of leukemia, reducible by v ..... For more information please see the full phenotype on the strain data sheet | ||
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