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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Cldn9nmf329
neuroscience mutagenesis facility, 329
MGI:3055084
Summary 2 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Cldn9nmf329/Cldn9nmf329 C57BL/6J-Cldn9nmf329/J MGI:4357857
cx2
Cldn9nmf329/Cldn9nmf329
Pou3f4del-J/Y
involves: C3HeB/FeJ * C57BL/6J MGI:4357858


Genotype
MGI:4357857
hm1
Allelic
Composition
Cldn9nmf329/Cldn9nmf329
Genetic
Background
C57BL/6J-Cldn9nmf329/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cldn9nmf329 mutation (1 available); any Cldn9 mutation (9 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Morphological defects at the basal turn of the cochlea in Cldn9nmf329/Cldn9nmf329 mice

hearing/vestibular/ear
• at the basal turn, the organ of corti is collapsed into a compact cell layer that lacks peracellular spaces and contains only two rows of outer hair cells instead of three as in wild-type mice
• many outer hair cells are missing from the reticular lamina and replaced by large polygonal cells unlike in wild-type mice
• at the basal turn, the organ of corti only has two rows of outer hair cells instead of three as in wild-type mice
• however, the number of outer hair cell rows in the apical turn is normal
• at P80, outer hair cells from the apex of the cochlea are missing from the first row
• in all three rows of the basal turn and the first two rows in the apical turn at P14
• in all three rows of the basal and apical turns at P80
• at P14 in the basal turn, outer hair cells from the first and second rows are missing due to degeneration
• at P14, the apical turn exhibits milder degeneration of outer hair cells from the first row
• degeneration of the hearing organ slows after P14 with some outer hair cells remaining at P80
• however, cultured explants of the organ of Corti do not exhibit degeneration
• routine examination for auditory function at 4-5 weeks of age revealed the mutant mice to have no auditory brainstem response at any frequency tested (J:87349)
• at P28, auditory brainstem response threshold is increased at high- and low-frequencies compared to in wild-type mice (J:152154)
• at P16, hearing loss is severe and deafness has an early onset (J:152154)
• the concentration of potassium ions in the perilymph is higher than in wild-type mice

nervous system
• at the basal turn, the organ of corti only has two rows of outer hair cells instead of three as in wild-type mice
• however, the number of outer hair cell rows in the apical turn is normal
• at P80, outer hair cells from the apex of the cochlea are missing from the first row
• in all three rows of the basal turn and the first two rows in the apical turn at P14
• in all three rows of the basal and apical turns at P80
• at P14 in the basal turn, outer hair cells from the first and second rows are missing due to degeneration
• at P14, the apical turn exhibits milder degeneration of outer hair cells from the first row
• degeneration of the hearing organ slows after P14 with some outer hair cells remaining at P80
• however, cultured explants of the organ of Corti do not exhibit degeneration

behavior/neurological
N
• mice exhibit normal balance on a rotating rod
• routine examination for auditory function at 4-5 weeks of age revealed the mutant mice to have no acoustic startle response at any frequency tested (J:87349)




Genotype
MGI:4357858
cx2
Allelic
Composition
Cldn9nmf329/Cldn9nmf329
Pou3f4del-J/Y
Genetic
Background
involves: C3HeB/FeJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cldn9nmf329 mutation (1 available); any Cldn9 mutation (9 available)
Pou3f4del-J mutation (1 available); any Pou3f4 mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
N
• mice do not exhibit loss of outer hair cells unlike in Cldn9nmf329 homozygotes





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last database update
05/07/2024
MGI 6.23
The Jackson Laboratory