Alhadeff J A
Clin Chim Acta. 1978 Nov 1;89(3):469-73. doi: 10.1016/0009-8981(78)90411-4.
The specific activity of several lysosomal hydrolases in normal and cystic fibrosis (CF) livers has been investigated. Neuraminidase activity with respect to two natural substrates (fetuin and bovine submaxillary mucin) is normal in CF livers. No significant differences were found between CF and normal livers in 4-methylumbelliferyl-alpha-D-mannosidase specific activities determined at three different pH values (4.3, 5.65 and 6.5) corresponding to lysosomal, golgi and cytosolic components of the enzyme, respectively. The specific activities of p-nitrophenyl-beta-D-mannosidase and 4-methylumbelliferyl-beta-N-acetylglucosaminidase were also both similar in CF and normal livers.
已经对正常肝脏和囊性纤维化（CF）肝脏中几种溶酶体水解酶的比活性进行了研究。CF肝脏中针对两种天然底物（胎球蛋白和牛颌下粘蛋白）的神经氨酸酶活性正常。在分别对应于该酶的溶酶体、高尔基体和胞质成分的三个不同pH值（4.3、5.65和6.5）下测定的4-甲基伞形酮基-α-D-甘露糖苷酶比活性，CF肝脏和正常肝脏之间未发现显著差异。对硝基苯基-β-D-甘露糖苷酶和4-甲基伞形酮基-β-N-乙酰氨基葡萄糖苷酶的比活性在CF肝脏和正常肝脏中也都相似。
