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Phenotypes Associated with This Genotype
Genotype
MGI:8375874
Allelic
Composition
Prps1tm1.1Xzl/Y
Genetic
Background
involves: 129 * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Prps1tm1.1Xzl mutation (0 available); any Prps1 mutation (21 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
• by 48 weeks of age, a decrease in the number of cochlear inner hair cells is seen in the basal turn of the cochlea
• by 48 weeks of age, a decrease in the number of cochlear outer hair cells is seen in the basal turn of the cochlea
• a small, but significant, increase in auditory threshold is detected at 32 kHz high frequencies at 4-12 weeks of age, which is further increased at 16-24 weeks of age
• by 48 weeks of age, significant hearing loss is detected at 8, 16 and 32 kHz
• mice show mild progressive hearing loss
• however, mice show normal inner ear development and no signs of vestibular dysfunction

homeostasis/metabolism

nervous system
• by 48 weeks of age, a decrease in the number of cochlear inner hair cells is seen in the basal turn of the cochlea
• by 48 weeks of age, a decrease in the number of cochlear outer hair cells is seen in the basal turn of the cochlea
• mice exhibit a decrease in spiral ganglion density and large number of empty spaces that are scattered throughout Rosenthals canal, indicating loss of spiral ganglion neurons in the apical, middle, and basal turns of the cochlea at 48 weeks of age

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
X-linked deafness 1 DOID:0111739 OMIM:304500
J:388624


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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last database update
06/09/2026
MGI 6.24
The Jackson Laboratory