Nakashima M, Kudoh T, Sukegawa K, Maruyama K, Orii T
Tohoku J Exp Med. 1986 Apr;148(4):365-71. doi: 10.1620/tjem.148.365.
The metabolism of [choline-methyl-14C]sphingomyelin in cultured skin fibroblasts from patients with different types of Niemann-Pick disease was measured 1 and 3 days after uptake from the media. The cell lines obtained from type A disease had more than 95% unhydrolyzed sphingomyelin in situ on day 3 while two cell lines obtained from type B had 36.3% and 43.3% unhydrolyzed sphingomyelin on day 3. The cell line derived from one patient with the transitory type disease had 48.1% unhydrolyzed sphingomyelin on day 3, and there was no significant difference in the sphingomyelinase activity measured in vitro or in degradation of sphingomyelin in situ between the type B and transitory type disease. In three cell lines from patients with type C disease, there was 18.5%, 29.6% and 31.1% unhydrolyzed sphingomyelin on day 3, which indicates that this type has a decreased ability to metabolize sphingomyelin. Cell from type E disease metabolized sphingomyelin normally.
在从培养基中摄取[胆碱 - 甲基 - 14C]鞘磷脂1天和3天后，测定了不同类型尼曼 - 匹克病患者培养的皮肤成纤维细胞中[胆碱 - 甲基 - 14C]鞘磷脂的代谢情况。来自A型疾病的细胞系在第3天原位有超过95%的未水解鞘磷脂，而来自B型的两个细胞系在第3天有36.3%和43.3%的未水解鞘磷脂。来自一名短暂型疾病患者的细胞系在第3天有48.1%的未水解鞘磷脂，并且在B型和短暂型疾病之间，体外测定的鞘磷脂酶活性或原位鞘磷脂降解方面没有显著差异。在来自C型疾病患者的三个细胞系中，第3天有18.5%、29.6%和31.1%的未水解鞘磷脂，这表明该类型代谢鞘磷脂的能力下降。来自E型疾病的细胞正常代谢鞘磷脂。
