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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID 		Cib2em1Xuzg
endonuclease-mediated mutation 2, Zhigang Xu
MGI:7612327
Summary 3 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
 		Cib2em1Xuzg/Cib2em1Xuzg C57BL/6-Cib2em1Xuzg MGI:7612334
ht2
 		Cib2em1Xuzg/Cib2+ C57BL/6-Cib2em1Xuzg MGI:7612581
cx3
 		Cib2em1Xuzg/Cib2em1Xuzg
Cib3em1Xuzg/Cib3em1Xuzg 		involves: C57BL/6 * C57BL/6N MGI:7612578


Genotype
MGI:7612334
hm1
Allelic
Composition		 Cib2em1Xuzg/Cib2em1Xuzg
Genetic
Background		 C57BL/6-Cib2em1Xuzg
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cib2em1Xuzg mutation (0 available); any Cib2 mutation (16 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
behavior/neurological
impaired balance ( J:345936 )
• mild impairment at 9 months of age but not at 6 months of age

hearing/vestibular/ear
abnormal inner hair cell stereociliary bundle morphology ( J:280151 )
• second and third row stereocilia are over-grown but the staircase architecture is maintained at P20
abnormal inner hair cell kinocilium morphology ( J:280151 )
• kinocilium is not retracted properly at P20
abnormal outer hair cell stereociliary bundle morphology ( J:280151 )
• disorganized P7 and more severe by P30
• fragmentation in some mice at P7
• at P8 the second row sterocilia are over-grown to the height close to the first row and the third row are largely retracted disrupting the staircase architecture
decreased outer hair cell stereocilia number ( J:280151 )
• some outer hair cells completely lose their stereocilia at P30
abnormal vestibular hair cell stereociliary bundle morphology ( J:345936 )
• striolar vestibular hair cell sterocilia in saccules are less organized at P30
decreased vestibular hair cell stereocilia number ( J:345936 )
• decreased numbers of striolar vestibular hair cell strereocilia in the saccules and utricles at P30
• further decreased in utricles at P90
abnormal cochlear outer hair cell physiology ( J:280151 )
• voltage-gated currents are slightly reduced at P7 but not at P1 or P4
abnormal hair cell mechanoelectric transduction ( J:280151 )
• at P7 mechanoelectric transduction currents are completely abolished in outer and inner hair cells
increased or absent threshold for auditory brainstem response ( J:280151 )
• increased threshold by P14 with click stimuli at 90 dB failing to elicit a response
• thresholds are also increased for pure tone stimuli

nervous system
abnormal inner hair cell stereociliary bundle morphology ( J:280151 )
• second and third row stereocilia are over-grown but the staircase architecture is maintained at P20
abnormal inner hair cell kinocilium morphology ( J:280151 )
• kinocilium is not retracted properly at P20
abnormal outer hair cell stereociliary bundle morphology ( J:280151 )
• disorganized P7 and more severe by P30
• fragmentation in some mice at P7
• at P8 the second row sterocilia are over-grown to the height close to the first row and the third row are largely retracted disrupting the staircase architecture
decreased outer hair cell stereocilia number ( J:280151 )
• some outer hair cells completely lose their stereocilia at P30
abnormal vestibular hair cell stereociliary bundle morphology ( J:345936 )
• striolar vestibular hair cell sterocilia in saccules are less organized at P30
decreased vestibular hair cell stereocilia number ( J:345936 )
• decreased numbers of striolar vestibular hair cell strereocilia in the saccules and utricles at P30
• further decreased in utricles at P90
abnormal cochlear outer hair cell physiology ( J:280151 )
• voltage-gated currents are slightly reduced at P7 but not at P1 or P4
abnormal hair cell mechanoelectric transduction ( J:280151 )
• at P7 mechanoelectric transduction currents are completely abolished in outer and inner hair cells

cellular
abnormal inner hair cell kinocilium morphology ( J:280151 )
• kinocilium is not retracted properly at P20




Genotype
MGI:7612581
ht2
Allelic
Composition		 Cib2em1Xuzg/Cib2+
Genetic
Background		 C57BL/6-Cib2em1Xuzg
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cib2em1Xuzg mutation (0 available); any Cib2 mutation (16 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
hearing/vestibular/ear
decreased vestibular hair cell stereocilia number ( J:345936 )
• in striolar vestibular hair cells in utricles at P30, but not as severe as in homozygous mice
• further decreased at P90

nervous system
decreased vestibular hair cell stereocilia number ( J:345936 )
• in striolar vestibular hair cells in utricles at P30, but not as severe as in homozygous mice
• further decreased at P90




Genotype
MGI:7612578
cx3
Allelic
Composition		 Cib2em1Xuzg/Cib2em1Xuzg
Cib3em1Xuzg/Cib3em1Xuzg
Genetic
Background		 involves: C57BL/6 * C57BL/6N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cib2em1Xuzg mutation (0 available); any Cib2 mutation (16 available)
Cib3em1Xuzg mutation (0 available); any Cib3 mutation (12 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
hearing/vestibular/ear
decreased vestibular hair cell stereocilia number ( J:345936 )
• starting at P15 in the striolar and extrastriolar regions of saccules and utricles and increasing in severity at P30 and P90
abnormal hair cell mechanoelectric transduction ( J:345936 )
• mechanoelectric transduction currents are completely abolished in both striolar and extrastriolar vestibular hair cells

behavior/neurological
impaired balance ( J:345936 )
• severe impairment at P30 in both the rotarod and swimming tests
circling ( J:345936 )
• at P30

nervous system
decreased vestibular hair cell stereocilia number ( J:345936 )
• starting at P15 in the striolar and extrastriolar regions of saccules and utricles and increasing in severity at P30 and P90
abnormal hair cell mechanoelectric transduction ( J:345936 )
• mechanoelectric transduction currents are completely abolished in both striolar and extrastriolar vestibular hair cells




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Send questions and comments to User Support. 		last database update
11/12/2024
MGI 6.24 		The Jackson Laboratory