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Poster Presentations
Day 3, June 24（Tue.）
Room P (Maesato East, Foyer, Ocean Wing)
- 3P-AM-17
Analysis of serine-related lipid changes in Niemann-Pick disease type C model mice using an improved analytical method
(1Grad. Sch. Pharm. Sci., Tohoku Univ., 2Dept. Pharm. Sci., Tohoku Univ. Hosp., 3Tohoku Univ. Grad. Sch. Med., 4INGEM., Tohoku Univ.)
oKeitaro Miyoshi1, Masamitsu Maekawa1,2,3,4, Mikiko Suzuki3,4, Nariyasu Mano1,2,3
Niemann-Pick disease type C (NPC) is an autosomal recessive intractable disorder caused by NPC1 or NPC2 which code cholesterol transporting proteins. Our laboratory has previously found an increase in new lipid N-palmitoyl-O-phosphocholine-serine (PPCS) in NPC patients' blood. In this study, we examined the spatiotemporal (space: organs and blood, time: weeks of age in mice) variation of serine-related lipids in NPC model mice, especially PPCS and sphingosylphosphocholine (SPC), which are candidate molecules for NPC biomarkers. We investigated the lipid extraction conditions to improve the PPCS recovery rate. The ion intensity of PPCS increased about 45-fold from the conventional method by using chloroform/methanol/water/acetic acid for lipid extraction, and after optimizing various conditions, PPCS and SPC were successfully isolated and quantified in liver, spleen, cerebellum, cerebrum, and plasma samples from NPC model mice. Although it was clear that PPCS and SPC were present in large amounts in the plasma of NPC patients, systemic analysis using model mice in this study revealed that they also accumulated in various organs. In addition, the rapid increase in accumulation in the spleen suggests that the possibility of overproduction or accumulation of PPCS and SPC in blood cells.