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ペロキシソームの適応的増殖の障害による音波曝露に対する過度脆弱性
Sedigheh Delmaghani1, Jean Defourny1, Asadollah Aghaie2
1Unité de Génétique et Physiologie de l'Audition, Institut Pasteur, 75015 Paris, France; UMRS 1120, Institut National de la Santé et de la Recherche Médicale (INSERM), 75015 Paris, France; Sorbonne Universités, UPMC Université Paris 06, Complexité du Vivant, 75005 Paris, France.
Cell
|November 7, 2015
まとめ
ペイヴァキンの欠乏は,聴覚系におけるペロキシソームの抗酸化防御を損なうことで聴覚障害を引き起こす. このタンパク質は 騒音による損傷から 頭毛細胞とニューロンを保護するために不可欠です
科学分野:
- 耳鼻喉科
- 細胞生物学
- 遺伝学
背景:
- ペイヴァキン (Pjvk) 欠乏症はヒトの異質性難聴とネズミの変異性聴覚現象を引き起こします.
- ペイバキンの機能は以前は知られず,聴覚の健康に対する役割も不明でした.
研究 の 目的:
- 聴覚系におけるペイバキンの機能を明らかにする.
- ペイバキン欠乏による聴覚障害の分子メカニズムを調査する.
主な方法:
- ペイバキン欠乏症 (Pjvk ((- -)) のマウスと人間の聴覚障害患者の分析
- 音声と電気刺激を含む聴覚検査
- ペロキシソームと酸化ストレスマーカーに焦点を当てた,頭組織のサブセルラー分析.
- 騒音曝露に対するPjvkの遺伝子発現分析
主要な成果:
- 耳の毛細胞と聴覚神経は音に非常に脆弱です.
- ペイヴァキンはペロキシソームと関連しており,酸化ストレス下でのその増殖に不可欠です.
- Pjvk-/-) コchleasは酸化ストレス,抗酸化防御機能の低下,そして異常なペロキシソームの兆候を示しています.
- 騒音への曝露は,野生型のマウスの聴覚細胞におけるPjvk発現とペロキシソームの増殖を増加させる.
結論:
- ペイヴァキンは,聴覚系におけるペロキシソームの抗酸化機能に不可欠です.
- ペイバキンの欠乏によるペロキシソームの活動障害は,騒音による聴覚障害に対する感受性の高まりにつながります.
- ペロキシソームの抗酸化能力は,騒音による損傷から聴覚系を保護します.
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