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Phenotypes Associated with This Genotype
Genotype
MGI:3700967
ht3
Allelic
Composition
Sod1tm1Cep/Sod1+
Genetic
Background
involves: 129S1/Sv * 129X1/SvJ * CD-1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Sod1tm1Cep mutation (0 available); any Sod1 mutation (47 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
• at 13 months, heterozygotes display a similar degree of IHC degeneration in the mid-base and hook region relative to homozygotes
• at 13 months, heterozygotes show a significantly greater OHC degeneration in the mid-base and hook region relative to wild-type mice, with no significant differences between hetero- and homozygous mutant mice
• at 13 months, heterozygotes show a significantly greater, though variable, OHC loss in the apex relative to homozygotes
• in response to 4-kHz tone bursts from 90 to 40 dB peak SPL, 13-mo-old heterozygotes exhibit variable ABR waveform morphology relative to wild-type mice
• at 4 kHz, heterozygotes with minimal hair cell loss display ABR peaks of similar morphology and amplitude to those of wild-type mice with maximal hair cell loss
• at 4 kHz, ABRs of heterozygotes with maximal hair cell loss are smaller and more poorly defined than those of homozygotes with the least hair cell loss
• at 13 months, heterozygotes display significantly elevated ABR thresholds at 16 and 32 kHz (but not at 8 kHz) relative to wild-type mice
• at 13 months, heterozygotes exhibit increased susceptibility to age-related hearing loss and cochlear hair cell loss, though to a lesser degree than homozygotes

nervous system
• at 13 months, heterozygotes display a similar degree of IHC degeneration in the mid-base and hook region relative to homozygotes
• at 13 months, heterozygotes show a significantly greater OHC degeneration in the mid-base and hook region relative to wild-type mice, with no significant differences between hetero- and homozygous mutant mice
• at 13 months, heterozygotes show a significantly greater, though variable, OHC loss in the apex relative to homozygotes


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
12/17/2024
MGI 6.24
The Jackson Laboratory