Beck M, Lücke R, Kresse H
Clin Chim Acta. 1984 Aug 15;141(1):7-15. doi: 10.1016/0009-8981(84)90161-x.
It had been suggested that Dyggve-Melchior-Clausen syndrome may be due to the deficiency of a specific sulfatase and/or a protease involved in proteoglycan degradation. The ability of Dyggve-Melchior-Clausen fibroblasts to endocytose and degrade 3H-leucine- and 35S-sulfate-labelled proteodermatan sulfate and 35S-sulfate-labelled proteokeratan sulfate, respectively, was therefore investigated. The turnover of cell-associated 35S-sulfate-labelled heparan sulfate was also followed. In all these experiments Dyggve-Melchior-Clausen fibroblasts behaved normally.
有人提出，迪格维-梅尔基奥尔-克劳森综合征可能是由于参与蛋白聚糖降解的特定硫酸酯酶和/或蛋白酶缺乏所致。因此，研究了迪格维-梅尔基奥尔-克劳森成纤维细胞分别内吞和降解3H-亮氨酸和35S-硫酸盐标记的蛋白皮肤素硫酸酯以及35S-硫酸盐标记的蛋白角质素硫酸酯的能力。还追踪了细胞相关的35S-硫酸盐标记的硫酸乙酰肝素的周转情况。在所有这些实验中，迪格维-梅尔基奥尔-克劳森成纤维细胞表现正常。
