Carrell R W, Jeppsson J O, Laurell C B, Brennan S O, Owen M C, Vaughan L, Boswell D R
Nature. 1982 Jul 22;298(5872):329-34. doi: 10.1038/298329a0.
The sequence of alpha 1-antitrypsin is in keeping with its role as a tissue scavenger of leukocyte elastase. Two abnormal variants commonly present in Europeans cause a deficiency that predisposes them to a progressive loss of lung elasticity. The nature of the reactive centre helps explain why cigarette smoking greatly accelerates the onset and severity of this degenerative process to give the disease emphysema.
α1-抗胰蛋白酶的序列与其作为白细胞弹性蛋白酶的组织清除剂的作用相符。欧洲人常见的两种异常变体导致一种缺陷,使他们易患肺弹性的渐进性丧失。反应中心的性质有助于解释为什么吸烟会极大地加速这种退行性过程的发生和严重程度,从而导致肺气肿。