Pancreatic secretory trypsin inhibitor as a diagnostic marker for adult-onset type II citrullinemia
Pancreatic secretory trypsin inhibitor as a diagnostic marker for adult-onset type II citrullinemia
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DOI：
10.1002/hep.510250519
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发表时间：
1997-05-01
期刊：
影响因子：
13.5
通讯作者：
Saheki, T
中科院分区：
文献类型：
--
作者：
Kobayashi, K;Horiuchi, M;Saheki, T
成人II型瓜氨酸血症的特点是肝脏中特异性发现乙酰氨基琥珀酸合成酶（ASS）蛋白缺乏。我们最近的研究使用差异信使RNA（mRNA）显示，所有II型瓜氨酸血症患者肝脏中人胰腺分泌型胰蛋白酶抑制剂（hPSTI）mRNA的表达显著增加。在目前的工作中，我们发现，hPSTI蛋白的浓度高于在II型患者的肝脏比对照组。众所周知，PSTI是一种分泌性蛋白，而且血清PSTI已被认为是胰腺和非胰腺疾病的标志物，因此我们测定了II型患者血液中的hPSTI水平，并将其与其他血清标志物如弹性蛋白酶1、胰蛋白酶、磷脂酶A（2）、甲胎蛋白、CA 19 -9和C反应蛋白（CRP）进行比较。我们发现血清hPSTI水平显着增加，而其他血清标志物无变化。这些结果表明，血清hPSTI是一个有用的诊断标志物成人发病的II型瓜氨酸血症。
Adult-onset type II citrullinemia is characterized by which deficiency of argininosuccinate synthetase (ASS) protein is found specifically in the liver of patients, Our recent study using differential messenger RNA (mRNA) display showed that the expression of human pancreatic secretory trypsin inhibitor (hPSTI) mRNA increases significantly in the liver of all type II patients tested. In the present work, we found that the concentration of hPSTI protein was higher in the liver of type II patients than controls. Because it is well known that PSTI is a secretory protein and because serum PSTI has been proposed as a marker of pancreatic and nonpancreatic diseases, we measured the hPSTI level in the blood of type II patients and compared it with other serum markers such as elastase 1, trypsin, phospholipase A(2), alpha-fetoprotein, CA19-9, and C-reactive protein (CRP). We found a significant increase in serum hPSTI level with no change in the other serum markers. These results suggest that serum hPSTI is useful as a diagnostic marker for adult-onset type II citrullinemia.