Herd J K, Hayhome B A, Tschida J, Forrest T
Proc Soc Exp Biol Med. 1976 Apr;151(4):642-9. doi: 10.3181/00379727-151-39277.
Bovine testicular hyaluronidase (endo-beta-N-acetyl hexosaminidase) has a significant corrective effect on cultured Hurler fibroblasts. Nonspecificity of this effect is indicated by its equally strong corrective effect on Hunter fibroblasts. Although all specimens of hyaluronidase also possessed iduronidase activity, a separate corrective effect could be attributed to the endo-N-acetyl hexosaminidase activity of at least one hyaluronidase (Wyeth M-151) for four reasons: (i) its very low content of iduronidase activity; (ii) a decrease in intracellular macromolecular mucopolysaccharides (believed to be largely dermatan sulfate) with a corresponding increase in intracellular and extracellular oligosaccharides; (iii) no measurable increase in iduronidase activity of hyaluronidase-treated cells despite near maximal correction; (iv) direct correlation between Hurler cell correction and hyaluronidase activity when enzymes of different strength were used at less than maximal correction.
牛睾丸透明质酸酶(内切-β-N-乙酰己糖胺酶)对培养的Hurler成纤维细胞有显著的校正作用。这种作用的非特异性体现在它对Hunter成纤维细胞也有同样强的校正作用。尽管所有透明质酸酶标本也都具有艾杜糖醛酸酶活性,但至少有一种透明质酸酶(惠氏M-151)的内切-N-乙酰己糖胺酶活性可归因于一种单独的校正作用,原因有四点:(i)其艾杜糖醛酸酶活性含量极低;(ii)细胞内大分子粘多糖(认为主要是硫酸皮肤素)减少,同时细胞内和细胞外寡糖相应增加;(iii)尽管校正接近最大值,但透明质酸酶处理的细胞中艾杜糖醛酸酶活性没有可测量的增加;(iv)当使用不同强度的酶且校正未达到最大值时,Hurler细胞校正与透明质酸酶活性之间存在直接相关性。