Burkart T, Wiesmann U N, Siegrist H P, Herschkowitz N N
Biochim Biophys Acta. 1981 Mar 18;673(3):351-8. doi: 10.1016/0304-4165(81)90466-9.
Net sulfatide synthesis, galactosylceramide sulfotransferase (EC 2.8.2.11) and arylsulfatase A (EC 3.1.6.1) activities were measured in two brain regions, cerebrum and cerebellum, of normal and jimpy mice during postnatal development. In normally myelinating mice, two phases of increasing rates of net sulfatide synthesis were observed, the first coinciding with oligodendrocyte proliferation and the second with myelination. Net sulfatide synthesis was quantitatively higher in the cerebellum than in the cerebrum. In both brain regions, the developmental patterns of net sulfatide synthesis were related to the activity patterns of both galactosylceramide sulfotransferase and arylsulfatase A. In jimpy mice, a neurological mutant showing hypomyelination in brain, the first phase of net sulfatide synthesis was preserved in both brain regions and galactosylceramide sulfotransferase and arylsulfatase A activities were normal up to 12 days. However, during the phase in which myelination occurred in controls, the net sulfatide synthesis in both brain regions of jimpy mice was zero or even negative. The sulfatide deficit was larger in the cerebellum than in the cerebrum. In both mutant brain parts, galactosylceramide sulfotransferase activity increased up to 12 days showing about 50% of the maximal activities observed in normal brain regions. Thereafter up to 15 days, enzyme activity decreased to about 25% of that of controls and remained low in both brain regions. The developmental patterns and the activities of arylsulfatase A were, however, normal in the cerebrum and cerebellum of jimpy mice. These results suggest that the enzyme activities and the developmental patterns of galactosylceramide sulfotransferase and arylsulfatase A as measured in vitro reflect to a high degree their functional activity in vivo. Furthermore, sulfatide degradation by arylsulfatase A seems to be important in regulating net sulfatide synthesis during normal and impaired myelination.
在出生后发育过程中，对正常小鼠和jimpy小鼠大脑的两个区域（大脑和小脑）进行了净硫脂合成、半乳糖基神经酰胺硫酸转移酶（EC 2.8.2.11）和芳基硫酸酯酶A（EC 3.1.6.1）活性的测定。在正常髓鞘形成的小鼠中，观察到净硫脂合成速率增加的两个阶段，第一个阶段与少突胶质细胞增殖同时发生，第二个阶段与髓鞘形成同时发生。小脑的净硫脂合成在数量上高于大脑。在两个脑区中，净硫脂合成的发育模式与半乳糖基神经酰胺硫酸转移酶和芳基硫酸酯酶A的活性模式相关。在jimpy小鼠中，一种在大脑中表现为髓鞘形成不足的神经学突变体，两个脑区净硫脂合成的第一阶段均得以保留，并且在12天之前半乳糖基神经酰胺硫酸转移酶和芳基硫酸酯酶A的活性均正常。然而，在对照组发生髓鞘形成的阶段，jimpy小鼠两个脑区的净硫脂合成均为零甚至为负。小脑的硫脂缺乏比大脑更大。在突变小鼠的两个脑区中，半乳糖基神经酰胺硫酸转移酶活性在12天之前增加，显示出正常脑区观察到的最大活性的约50%。此后直到15天，酶活性降至对照组的约25%，并且在两个脑区中均保持较低水平。然而，jimpy小鼠大脑和小脑的芳基硫酸酯酶A的发育模式和活性是正常的。这些结果表明，体外测定的半乳糖基神经酰胺硫酸转移酶和芳基硫酸酯酶A的酶活性和发育模式在很大程度上反映了它们在体内的功能活性。此外，在正常和受损髓鞘形成过程中，芳基硫酸酯酶A对硫脂的降解似乎在调节净硫脂合成中起重要作用。
