Herrmann K, Schulze E, Haustein U F, Böhme H J, Ziegler V
Arch Dermatol Res. 1987;279(5):299-302. doi: 10.1007/BF00431221.
In 14 patients with progressive systemic sclerosis (PSS) the activities of acid lysosomal glycosidases (alpha-, beta-galactosidase, beta-glucosidase, beta-glucuronidase, and beta-N-acetyl-glucosaminidase) were determined fluorometrically in serum, leukocytes, and skin tissue. The beta-galactosidase was the only enzyme which exhibited a significantly elevated activity in PSS serum and skin but not leukocytes, as compared to the control. The activity patterns of the studied glycosidases in serum were similar to those found in skin, but differ from the distribution of glycosidase activities in leukocytes. In cultured dermal fibroblasts derived from PSS patients, an elevated intracellular activity of beta-galactosidase was detected. These results suggest that the increased beta-galactosidase activity in the serum originates from the skin fibroblasts.
对14例进行性系统性硬化症（PSS）患者，采用荧光法测定了其血清、白细胞及皮肤组织中酸性溶酶体糖苷酶（α-、β-半乳糖苷酶、β-葡萄糖苷酶、β-葡萄糖醛酸酶和β-N-乙酰氨基葡萄糖苷酶）的活性。与对照组相比，β-半乳糖苷酶是PSS患者血清和皮肤中唯一活性显著升高而白细胞中未升高的酶。所研究糖苷酶在血清中的活性模式与皮肤中的相似，但与白细胞中糖苷酶活性的分布不同。在源自PSS患者的培养真皮成纤维细胞中，检测到β-半乳糖苷酶的细胞内活性升高。这些结果表明，血清中β-半乳糖苷酶活性的增加源自皮肤成纤维细胞。
