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Allele Symbol Allele Name Allele ID |
Sod1tm1Cep targeted mutation 1, Cephalon MGI:1857872 |
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| Summary |
4 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• pre-B colony forming units are decreased compared to in wild-type mice
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• modest
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• at 2, 7, and 17-19 months, homozygotes show a significant increase in age-related baso-apical progression of hair cell (HC) loss relative to age-matched wild-type mice, with a greater loss of OHCs than IHCs within each age group
• at 17 months, homozygotes display a near-complete loss of both OHCs and IHCs whereas wild-type mice show massive OHC loss with largely intact IHCs
• HC loss is prominent at 17-19 months, with a high degree of variability among individual cochleas
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• at 2 months, homozygotes exhibit ~20-80% IHC loss in the basal 30% of the cochlea whereas wild-type mice show >20% IHC loss only in the basal-most 5% of the cochlea
(J:57435)
• between 2-7 months, IHC loss reaches 100% in the extreme base for both wild-type and mutant mice; however, homozygotes show a significantly greater IHC in the region 30-80% of the distance from the apex
(J:57435)
• at 13 months, homozygotes display significant IHC degeneration in the mid-base and hook region relative to wild-type mice, with no significant differences between hetero- and homozygous mutant mice
(J:118910)
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• at 2 months, homozygotes exhibit 20-100% OHC loss in the basal half of the cochlea whereas wild-type mice show only 0-50% OHC loss in the basal half; a similar OHC loss of <20% is noted in the apical half for both genotypes
(J:57435)
• at 7 months, OHC loss progresses to the apical half of the cochlea in mutant mice but remains confined to the basal half in wild-type mice
(J:57435)
• at 17-19 months, homozygotes exhibit 70-100% OHC loss throughout the cochlea relative to ~30-80% in wild-type mice
(J:57435)
• at 13 months, homozygotes display 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
(J:118910)
• at 13 months, homozygotes show a less pronounced OHC loss in the apical half relative to wild-type or heterozygous mice
(J:118910)
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• at 2 months, some pillar cells are lost
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• at 13 months, the organ of Corti is severely degenerated
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• in response to 4-kHz tine bursts from 90 to 40 dB peak SPL, even 13-mo-old homozygotes with minimal hair cell loss display poorly defined ABR peaks relative to age-matched wild-type mice with maximal hair cell loss
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• prior to broad-band noise exposure (4.0-45.0 kHz at 110 dB SPL for 1 hr), 5-13-wk-old homozygotes display a minor elevation of ABR thresholds (0-7 dB) at all test frequencies tested relative to age-matched wild-type mice
(J:56971)
• specifically, pre-exposed 5-13-wk-old homozygotes show an ABR threshold elevation of 6-7 dB at 5 and 10 kHz, but not at 20 or 40 kHz
(J:56971)
• pre-exposed 5-13-wk-old homozygotes and wild-type mice show a similar elevation of ABR thresholds of ~20-50 dB at 40 kHz
(J:56971)
• at 13 months, homozygotes display significantly elevated ABR thresholds at 8-, and 16- and 32-kHz relative to wild-type mice
(J:118910)
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• at 13 months, homozygotes exhibit increased susceptibility to age-related hearing loss and cochlear hair cell loss relative to wild-type mice
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• at 1 day after broad-band noise exposure (4.0-45.0 kHz at 110 dB SPL for 1 hr), homozygotes show a significantly greater acute ABR threshold elevation of 8 dB at 20 kHz relative to wild-type mice
• at day 14 after noise-exposure, homozygotes exhibit a significantly greater permanent threshold shift (PTS) only at 5 kHz (14 dB) and 40 kHz (6 dB) relative to wild-type mice
• overall, acute noise-exposed homozygotes show an additional PTS of ~10 dB at all test frequencies relative to wild-type mice, based on 28-day threshold data
• at 28 days after noise exposure, average cochleograms show no correlation between hair cell loss and homozygosity for the targeted gene
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• at 2, 7, and 17-19 months, homozygotes show a significant increase in age-related baso-apical progression of hair cell (HC) loss relative to age-matched wild-type mice, with a greater loss of OHCs than IHCs within each age group
• at 17 months, homozygotes display a near-complete loss of both OHCs and IHCs whereas wild-type mice show massive OHC loss with largely intact IHCs
• HC loss is prominent at 17-19 months, with a high degree of variability among individual cochleas
|
|
• at 2 months, homozygotes exhibit ~20-80% IHC loss in the basal 30% of the cochlea whereas wild-type mice show >20% IHC loss only in the basal-most 5% of the cochlea
(J:57435)
• between 2-7 months, IHC loss reaches 100% in the extreme base for both wild-type and mutant mice; however, homozygotes show a significantly greater IHC in the region 30-80% of the distance from the apex
(J:57435)
• at 13 months, homozygotes display significant IHC degeneration in the mid-base and hook region relative to wild-type mice, with no significant differences between hetero- and homozygous mutant mice
(J:118910)
|
|
• at 2 months, homozygotes exhibit 20-100% OHC loss in the basal half of the cochlea whereas wild-type mice show only 0-50% OHC loss in the basal half; a similar OHC loss of <20% is noted in the apical half for both genotypes
(J:57435)
• at 7 months, OHC loss progresses to the apical half of the cochlea in mutant mice but remains confined to the basal half in wild-type mice
(J:57435)
• at 17-19 months, homozygotes exhibit 70-100% OHC loss throughout the cochlea relative to ~30-80% in wild-type mice
(J:57435)
• at 13 months, homozygotes display 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
(J:118910)
• at 13 months, homozygotes show a less pronounced OHC loss in the apical half relative to wild-type or heterozygous mice
(J:118910)
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• at 13 months, homozygotes show a significantly greater loss of spiral ganglion cells than age-matched or 2-month-old wild-type mice, with a higher degree of degeneration observed in the base than in the apex
• unlike eighth nerve fiber survival, survival of spiral ganglion cells is not compromised by age
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• at 13 months of age, both homozygotes and wild-type mice display significantly fewer eighth nerve fibers than 2-month-old wild-type mice, with significantly greater loss in aged homozygotes than in aged wild-type mice
• similar to hair cell loss, eighth nerve fibers in the basal half are more vulnerable to aging and ROS-mediated damage than fibers in the apical half
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• at 13 months, heterozygotes display a similar degree of IHC degeneration in the mid-base and hook region relative to homozygotes
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• 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
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• 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
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• at 13 months, heterozygotes display significantly elevated ABR thresholds at 16 and 32 kHz (but not at 8 kHz) relative to wild-type mice
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• at 13 months, heterozygotes exhibit increased susceptibility to age-related hearing loss and cochlear hair cell loss, though to a lesser degree than homozygotes
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• at 13 months, heterozygotes display a similar degree of IHC degeneration in the mid-base and hook region relative to homozygotes
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• 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
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• the number of committed progenitor cells in the bone marrow is decreased compared to in wild-type mice
• however, the number of primitive progenitor numbers are normal
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• 75-fold
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• mice exhibit hypocellular bone marrow compared with wild-type mice
• however, the number of primitive progenitor numbers are normal
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• pre-B colony forming units are decreased 75-fold compared to in wild-type mice
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• lymphocytes exhibit immature nuclear chromatin unlike wild-type mice
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• mice develop zonal microvesicular hepatic steatosis unlike wild-type mice
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• primary liver cells generate increased levels of superoxide compared with wild-type cells
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• bone marrow exhibits an increase in fat cells compared to in wild-type mice
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• lymphocytes exhibit immature nuclear chromatin unlike wild-type mice
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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 04/30/2024 MGI 6.23 |
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