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空气中的自发积和与年龄相关的纤维性肺病
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
概括
酸性哺乳动物酶 (AMCase) 对于清除肺中的环境素至关重要. AMCase 缺乏导致小鼠胆积累,肺纤维化和过早死亡,这表明它在肺部健康中起作用.
科学领域:
- 肺免疫学
- 分子生物学
- 生物化学
背景情况:
- 是一种广泛存在的多糖体,由酶降解.
- 脊椎动物拥有基因酶,但它们的哺乳动物生理作用仍然基本不明.
- 酸性哺乳动物胆酶 (AMCase) 是由肺上皮细胞分泌的.
研究的目的:
- 研究AMCase在哺乳动物肺生理和肺纤维化中的作用.
- 确定AMCase缺乏对气道胆清除和肺部健康的影响.
- 研究肺部疾病中素积累和纤维化通路之间的联系.
主要方法:
- 产生和分析AMCase缺乏的小鼠.
- 气道胺聚合物水平和亲纤维细胞因子表达的评估.
- 对肺部纤维化组织进行细胞病理分析.
- 来自AMCase缺乏小鼠的上皮细胞的基因组丰富分析.
主要成果:
- 缺乏AMCase的小鼠出现过早的发病率和死亡率.
- 在缺陷的小鼠的呼吸道中观察到胺聚合物和亲纤维细胞因子的积累.
- 缺乏AMCase的小鼠发生了自发性肺纤维化,这种情况是可逆的.
- 来自缺陷小鼠的上皮细胞显示与纤维化相关的基因表达与细胞应激相关.
- 在患有其他形式肺纤维化的小鼠和患有间歇性肺病的人类的呼吸道中也发现了多余的基聚合物.
结论:
- AMCase对于气道清除和预防自发性肺纤维化至关重要.
- 在表皮细胞功能障碍的肺部疾病中,改变素清除会加剧纤维化途径.
- 恢复肺酶活性可能是肺纤维化的治疗策略.
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