Caspase-3-deficiency induces hyperplasia of supporting cells and degeneration of sensory cells resulting in the hearing loss
| Autor: | Hiroshi Mori, Yasuhiro Yoshikawa, Keisuke Kuida, Takashi Momoi, Keiichi Ichimura, Koko Urase, Toshihiro Takizawa, Miho Suga, Kazusaku Kamiya, Katsumasa Takahashi |
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| Rok vydání: | 2001 |
| Předmět: |
Pathology
medicine.medical_specialty Programmed cell death Hearing Loss Sensorineural Stereocilia (inner ear) Apoptosis Gene Expression Regulation Enzymologic Mice Audiometry otorhinolaryngologic diseases medicine Animals Inner ear Cilia Molecular Biology Caspase Cochlea Mice Knockout Hair Cells Auditory Inner Hyperplasia biology Caspase 3 General Neuroscience Gene Expression Regulation Developmental medicine.disease Cell biology Mice Inbred C57BL medicine.anatomical_structure Organ of Corti Caspases Nerve Degeneration biology.protein sense organs Neurology (clinical) Hair cell Spiral Ganglion Developmental Biology |
| Zdroj: | Brain Research. 894:359-367 |
| ISSN: | 0006-8993 |
| DOI: | 10.1016/s0006-8993(01)02123-0 |
| Popis: | Caspase-3 is one of the cystein proteases that play essential roles in programmed cell death. As such, brain development is profoundly affected by caspase-3-deficiency, resulting in hyperplasia and abnormal cell organization (Kuida et al., Nature 1996;384:368-372). In the present study, we used caspase-3 (-/-) mice to show that caspase-3 deficiency results in severe hearing loss, hyperplasia of supporting cells and degeneration of sensory hair cells. The greater epithelial ridge, a remnant of the primordial organ of Corti, persists throughout all of the turns of cochlea in 2-week-old caspase-3 (-/-) mice, which indicates that the morphology of the cochlea is immature. The number of border cells, that develop from the greater epithelial ridge and are one of the supporting cells of the inner hair cell, increase significantly in both 2- and 5-week-old caspase-3 (-/-) mice. On the other hand, abnormal fused stereocilia can be seen in both 2- and 5-week-old caspase-3 (-/-) mice, and disarrangement and loss of sensory hair cells are observed in 5-week-old caspase-3 (-/-) mice. Taken together, both hyperplasia and degeneration occur simultaneously in the inner ear of the caspase-3 (-/-) mice, suggesting that caspase-3-dependent apoptosis is necessary for the development and formation of a properly functioning auditory system in mammals. |
| Databáze: | OpenAIRE |
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