Jeppsson J O, Eriksson S
Biochim Biophys Acta. 1985 Sep 20;831(1):30-3. doi: 10.1016/0167-4838(85)90145-1.
The alpha 1-antitrypsin from the liver of a subject with alpha i-antitrypsin deficiency was purified and subjected to automated Edman degradation. The N-terminal amino acid sequence from position 1 to 12 was identical to that in plasma alpha 1-antitrypsin, type Z. This result precludes that the intrahepatic accumulation of Z alpha 1-antitrypsin is due to a defective removal of a signal peptide.
从患有α1 -抗胰蛋白酶缺乏症患者肝脏中提取的α1 -抗胰蛋白酶被纯化，并进行自动埃德曼降解。从第1位到第12位的N端氨基酸序列与血浆中Z型α1 -抗胰蛋白酶的序列相同。这一结果排除了Z型α1 -抗胰蛋白酶在肝内蓄积是由于信号肽去除缺陷所致的可能性。
