Iminosugar-based inhibitors of glucosylceramide synthase increase brain glycosphingolipids and survival in a mouse model of Sandhoff disease
基于亚氨基糖的葡糖基神经酰胺合成酶抑制剂可增加桑德霍夫病小鼠模型的脑糖鞘脂含量并提高其存活率。
期刊:PLoS One
影响因子:2.6
doi:10.1371/journal.pone.0021758
Ashe, Karen M; Bangari, Dinesh; Li, Lingyun; Cabrera-Salazar, Mario A; Bercury, Scott D; Nietupski, Jennifer B; Cooper, Christopher G F; Aerts, Johannes M F G; Lee, Edward R; Copeland, Diane P; Cheng, Seng H; Scheule, Ronald K; Marshall, John
