Pentchev P G, Gal A E, Wong R, Morrone S, Neumeyer B, Massey J, Kanter R, Sawitsky A, Brady R O
Biochim Biophys Acta. 1981 Sep 24;665(3):615-8. doi: 10.1016/0005-2760(81)90279-4.
Metabolically inert L-[1-14C]glucosylceramide is stored predominantly in the liver after intravenous administration to mice. The half-time of this glycolipid analogue in the liver is 3.5 days and its clearance occurs predominantly via the bile. Within the limited number of Gaucher specimens available for examination very high levels of glucosylceramide were found in the bile of one patient and in the liver of two patients with biliary obstruction. The question of a possible relationship between biliary excretion of glycolipid and the pathogenesis of Gaucher's disease will require further studies.
代谢惰性的L-[1-¹⁴C]葡萄糖神经酰胺经静脉注射给小鼠后，主要储存在肝脏中。这种糖脂类似物在肝脏中的半衰期为3.5天，其清除主要通过胆汁进行。在有限数量可供检查的戈谢病标本中，在一名患者的胆汁以及两名患有胆道梗阻患者的肝脏中发现了非常高水平的葡萄糖神经酰胺。糖脂的胆汁排泄与戈谢病发病机制之间可能存在的关系问题需要进一步研究。
