Benjamins J A, Fitch J, Radin N S
Brain Res. 1976 Feb 6;102(2):267-81. doi: 10.1016/0006-8993(76)90882-9.
Analogs of ceramide which inhibit galactocerebrosidase also demyelinate or inhibit myelination in organ cultures of rat cerebellum. The potency of the analogs in culture correlated with their effectiveness as inhibitors of cerebrosidase, but not with their effectiveness as inhibitors of galactosyl transferase. The most effective compound was the decanoyl amide of 3-phenyl-2-amino-1,3-propanediol with erythroconformation. Stimulators of cerebrosidase also demyelinated cultures. With both groups of compounds, myelin sheaths became distorted, then broke into lipid droplets. Axons were preserved, but neurons showed some nuclear changes and granularity. Metabolic studies with the most effective inhibitor showed that glucose incorporation into cerebroside and other alkali-stable lipids was initially depressed compared to proteins and total lipids.
抑制半乳糖脑苷脂酶的神经酰胺类似物在大鼠小脑器官培养物中也会导致脱髓鞘或抑制髓鞘形成。这些类似物在培养物中的效力与其作为脑苷脂酶抑制剂的有效性相关，但与其作为半乳糖基转移酶抑制剂的有效性无关。最有效的化合物是具有赤型构象的3-苯基-2-氨基-1,3-丙二醇的癸酰酰胺。脑苷脂酶的刺激剂也会使培养物脱髓鞘。对于这两组化合物，髓鞘都会变形，然后分裂成脂滴。轴突得以保留，但神经元显示出一些核变化和颗粒状。对最有效的抑制剂进行的代谢研究表明，与蛋白质和总脂质相比，最初葡萄糖掺入脑苷脂和其他碱稳定脂质的量会降低。
