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KMID : 0620920200520010016
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Experimental & Molecular Medicine
2020 Volume.52 No. 1 p.16 ~ p.16
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Degradation and modification of cochlear gap junction proteins in the early development of age-related hearing loss
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Tajima Shori
Danzaki Keiko
Ikeda Katsuhisa
Kamiya Kazusaku
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Abstract
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Age-related hearing loss (ARHL) is the progressive, bilateral loss of high-frequency hearing in elderly people. Mutations in GJB2, encoding the cochlear gap junction protein connexin26 (Cx26), are the most frequent cause of hereditary deafness; however, a common molecular pathology between ARHL and GJB2-related hearing loss has not been reported. Here, we investigated the quantitative change in expression and molecular pathology of Cx26 in ARHL. We used C57BL/6J mice as a model of ARHL. Hearing levels that were evaluated by auditory brainstem response thresholds increased gradually between 4 and 32 weeks of age and increased sharply at 36 weeks. Gap junctions in the cochleae of 4-week-old mice had linear plaques along cell?cell junction sites. In contrast, the cochleae from 32-week-old mice had significantly shorter gap junctions. Severe hair cell loss was not observed during this period. Based on western blotting, Cx26 and connexin30 (Cx30) levels were significantly decreased at 32 weeks compared with 4 weeks.
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KEYWORD
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Experimental models of disease, Protein quality control
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