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空気中の自発的なキチンの蓄積と,年齢に関連する線維性肺疾患
Steven J Van Dyken1, Hong-Erh Liang1, Ram P Naikawadi1
1Department of Medicine, University of California, San Francisco, San Francisco, CA 94143, USA.
Cell
|April 22, 2017
まとめ
酸性哺乳類キチナーゼ (AMCase) は,肺内の環境キチンの除去に不可欠です. AMCase欠乏はチチンの蓄積,肺線維症,およびマウスの早死につながり,肺の健康に作用することを示唆しています.
科学分野:
- 肺免疫学
- 分子生物学
- 生物化学
背景:
- 広範囲に存在する多糖質であるキチンはキチナゼによって分解される.
- 脊椎動物にはキチナゼがあるが,哺乳類の生理学的役割はほとんど不明である.
- 酸性哺乳類キチナーゼ (AMCase) は肺上皮細胞によって分泌される.
研究 の 目的:
- 哺乳類の肺生理と肺線維症におけるAMCaseの役割を調査する.
- 呼吸道キチンのクリアランスと肺の健康に対するAMCase欠乏の影響を決定する.
- 肺疾患におけるキチンの蓄積と 繊維性の経路の関係を調べる
主な方法:
- AMCase欠乏したマウスの生成と分析
- 呼吸道キチンポリマーの濃度とプロフィブロティック・サイトカインの発現の評価
- 肺組織を組織病理学的に分析して 線維症を検出する
- AMCase欠乏したマウスの上皮細胞の遺伝子セット濃縮分析.
主要な成果:
- AMCase欠乏したマウスは早発症と早死を示した.
- 欠乏したマウスの呼吸道にキチンポリマーとプロフィブロティック・サイトカインの蓄積が観察された.
- AMCase欠乏症のマウスは自発的な肺線維症を発症し,それは逆転することができました.
- 欠乏したマウスの上皮細胞は,細胞ストレスに関連した線維症関連遺伝子発現を示した.
- 過剰なキチンポリマーは,他の形態の肺線維症のマウスの呼吸道や,インタースティシャル肺疾患のヒトでも発見されました.
結論:
- AMCaseは呼吸道キチンのクリアランスと自発性肺線維症の予防に不可欠です.
- チチンのクリアランスの変化は,上皮細胞機能不全による肺疾患における線維性経路を悪化させる.
- 肺キチナーゼの活性回復は肺線維症の治療策である可能性があります.
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