Rushton A R, Dawson G
Clin Chim Acta. 1977 Oct 1;80(1):133-9. doi: 10.1016/0009-8981(77)90272-8.
The apparent low level of synthetic substrate beta-D-galactosidase activity in liver from patients with the Hurler-Hunter syndrome is caused by the inhibitory effect of accumulated glycosaminoglycans. We have demonstrated complete inhibition of GM1 ganglioside beta-galactosidase activity in vitro by both heparan sulfate and dermatan sulfate, but the effect on lactosylceramide and galactosylceramide hydrolysis was less marked. In contrast, lysosomal neuraminidase activity in vitro was enhanced by the addition of glycosaminoglycans. These observations are discussed in relationship to the observed storage pattern of glycosphingolipids in liver from patients with mucopolysaccharidoses.
患有胡尔勒-亨特综合征患者肝脏中合成底物β-D-半乳糖苷酶活性明显较低，是由积累的糖胺聚糖的抑制作用导致的。我们已经证明，硫酸乙酰肝素和硫酸皮肤素在体外均可完全抑制GM1神经节苷脂β-半乳糖苷酶的活性，但对乳糖基神经酰胺和半乳糖基神经酰胺水解的影响则不太明显。相反，在体外添加糖胺聚糖可增强溶酶体神经氨酸酶的活性。结合黏多糖贮积症患者肝脏中糖鞘脂的观察到的储存模式，对这些观察结果进行了讨论。
