Joseph病黄氏家系患者皮肤成纤维细胞组织化学研究

本文用细胞化学方法,对Joseph病黄氏家系患者(2例)的皮肤成纤维细胞的DNA、糖原、脂类的含量和SDH、LDH、GDH、ACPase及过氧化物酶的活性进行了观察和显微分光光度计定量测定。并与非神经性疾患患者(1例)对照,发现①Joseph病组培养的皮肤成纤维细胞的DNA含量、GDH和过氧化物酶的活性明显低于对照组;②Joseph病组皮肤成纤维细胞的LDH、ACPase活性及糖原的含量显著高于对照组。此外,也看到少量中性脂肪小滴存在于1例Joseph病患者培养的皮肤成纤维细胞质里。本结果表明上述变化可能与Joseph病的病理发生有关。

CYTOCHEMICAL STUDIES ON CULTURED SKIN FIBROBLASTS IN THE PATIENTS OF HUANG'S FAMILY OF JOSEPH DISEASE

The contents of DNA, glycogen, lipid, and the activities of SDH, LDH, GDH, ACPase and peroxidase of the cultured skin fibroblasts of the patients from Huang's family of Joseph disease(2 cases)and one case of control subject without neurological damage were observed cytochemically and measured microspectrophotometrically. The results showed that; (1) the content of DNA,the activities of GDH and peroxidase were apparently lower,and(2)the activities of LDH, ACPase, and the content of glycogen were higher in the cultured skin fibroblasts of the patients from Huang's family with Joseph disease compared with control subject. Besides, some neutral fat droplets were also observed in the cytoplama of the fibroblasts of the Joseph disease. The results indicated that there existed some degenerarative phenomena in the cultured skin fibroblasts of the patients from Huang's family of Joseph disease, and the changes mentioned above may be involved in the pathogenesis of Joseph disease.