Z α-1 antitrypsin deficiency and the endoplasmic reticulum stress response

• 作 者: Catherine M Greene Noel G McEl
• 机 构: RespiratoryResearchDivision
• 出 处: 世界胃肠药理与治疗学杂志：英文版（电子版）
• 发表日期: 2010年5期
• 关键词: α-1 ANTITRYPSIN Unfolded protein response Endoplasmic reticulum stress APOPTOSIS
• 基金项目: Supported by The U.S. Alpha One Foundation,the Health Research Board of Ireland,the Medical Research Charities Group,the Programmes for Research in Third Level Institutes administered by the Higher Ed

The serine proteinase inhibitor α-1 antitrypsin(AAT) is produced principally by the liver at the rate of 2 g/d.It is secreted into the circulation and provides an antiprotease protective screen throughout the body but most importantly in the lung,where it