沃尔曼病中酸性酯酶的特征
Characteristics of acid esterase in Wolman's disease.
作者信息
Maehira F, Nakada F, Hokama T
出版信息
Biochem Med. 1984 Dec;32(3):322-30. doi: 10.1016/0006-2944(84)90037-1.
The characteristics of acid esterase from the patient with Wolman's disease, a rare familial lipidosis, were studied. Enzymatic analysis as well as mineral analysis were performed on the patient's liver, spleen, and adrenal glands. Acid esterase was low in the patient's leucocytes and other affected tissues. Further enzymatic study with subcellular fractions of the liver in both patient and control subject revealed that acid esterase was mostly localized in the membrane of lysosomes. The lysosomal esterase was unaffected by Ca2+, Mg2+, EDTA, E600 (microsomal esterase inhibitor), and it was less inhibited by NaCl than other fractions. Studies with those inhibitors showed that acid esterase has different properties compared to other lipases, such as lipoprotein lipase, adipose tissue lipase, and hepatic microsomal lipase. Studies with inhibitors also gave a negative view on a possible suppressive interaction of the high content of calcium in the target organs with acid esterase in Wolman's disease.
对患有罕见家族性脂质沉积症——沃尔曼病患者的酸性酯酶特性进行了研究。对患者的肝脏、脾脏和肾上腺进行了酶分析以及矿物质分析。患者白细胞和其他受影响组织中的酸性酯酶含量较低。对患者和对照对象肝脏的亚细胞组分进行的进一步酶学研究表明，酸性酯酶主要定位于溶酶体膜。溶酶体酯酶不受Ca2+、Mg2+、EDTA、E600（微粒体酯酶抑制剂）的影响，与其他组分相比，其受NaCl的抑制作用较小。使用这些抑制剂的研究表明，酸性酯酶与其他脂肪酶，如脂蛋白脂肪酶、脂肪组织脂肪酶和肝微粒体脂肪酶相比，具有不同的特性。使用抑制剂的研究也对沃尔曼病靶器官中高钙含量与酸性酯酶之间可能存在的抑制性相互作用给出了否定观点。
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