Onishi T, Suzue J, Nishikawa K, Muramatsu M, Miyao M
Eur J Pediatr. 1981 Sep;137(1):17-21. doi: 10.1007/BF00441163.
Copper and zinc concentrations, and the nature of the copper- and zinc-binding proteins, were studied using tissues from a Menkes patient who had been given intravenous infusions of cupric acetate. The liver and brain copper contents were lower than in an untreated, non-Menkes control, and the spleen, intestine and kidney showed higher copper concentrations than control tissues. Zinc concentrations in all the organs (except the kidneys) from the Menkes patient were slightly lower than those the control child. Using Sephadex G-75 column chromatography of cytosols (105,000 x g supernatant), three copper- and zinc-containing peaks were eluted. In all the Menkes tissues studied, copper was prominent in peak 3. On the other hand, peak 3 was the smallest and peak 1 was the largest in the control tissues. Zinc predominated in peak 1 in both Menkes and control tissues, except for Menkes kidney. In this tissue peak 3 was again the largest. The copper- and zinc-binding material in peak 3 fractions from Menkes kidney was chromatographed on DEAE-cellulose columns. Three copper- and zinc-containing peaks were observed at the same positions as metallothionein from human adult kidneys.
使用一名接受过醋酸铜静脉输注的门克斯病患者的组织，研究了铜和锌的浓度以及铜结合蛋白和锌结合蛋白的性质。该患者肝脏和大脑中的铜含量低于未经治疗的非门克斯病对照，而脾脏、肠道和肾脏中的铜浓度高于对照组织。门克斯病患者所有器官（肾脏除外）中的锌浓度略低于对照儿童。使用葡聚糖G-75柱对胞质溶胶（105,000×g上清液）进行色谱分析，洗脱得到三个含铜和锌的峰。在所研究的所有门克斯病组织中，铜在峰3中最为突出。另一方面，在对照组织中，峰3最小，峰1最大。在门克斯病组织和对照组织中，除门克斯病患者的肾脏外，锌在峰1中占主导地位。在该组织中，峰3再次最大。对门克斯病患者肾脏峰3组分中的铜结合物质和锌结合物质在DEAE-纤维素柱上进行色谱分析。在与成人肾脏金属硫蛋白相同的位置观察到三个含铜和锌的峰。
