Phillips N C, Thorpe R
Clin Chim Acta. 1978 Feb 1;83(1-2):93-8. doi: 10.1016/0009-8981(78)90211-5.
The levels of three lysosomal glycosidase, alpha-D-mannosidase, a-L-fucosidase and beta-D-hexosaminidase have been determined in normal hair roots and in hair roots obtained from a patient with mannosidosis. The most active glycosidase in normal hair roots was beta-D-hexosaminidase, followed by alpha-L-fucosidase and alpha-D-mannosidase. There was no alpha-D-mannosidase activity in the hair roots of the patient with mannosidosis. The significance of these results is discussed in relation to the detection of lysosomal storage diseases.
已测定了正常发根以及来自一名甘露糖苷贮积症患者的发根中三种溶酶体糖苷酶(α-D-甘露糖苷酶、α-L-岩藻糖苷酶和β-D-己糖胺酶)的水平。正常发根中活性最高的糖苷酶是β-D-己糖胺酶,其次是α-L-岩藻糖苷酶和α-D-甘露糖苷酶。甘露糖苷贮积症患者的发根中没有α-D-甘露糖苷酶活性。结合溶酶体贮积病的检测对这些结果的意义进行了讨论。