Fig. 7: PFIC1 patients present with decreased plasma concentrations of choline and its metabolites.
From: Intestinal Atp8b1 dysfunction causes hepatic choline deficiency and steatohepatitis
Fasting plasma was collected from PFIC1 patients with (n = 12) or without LTx (n = 10), other cholestatic patients with (n = 20) or without LTx (n = 27), and age-matched control subjects (n = 31). Demographic information of the patients is summarized in Table 2 and Supplementary Tables 2 and 3. a–c Plasma concentration of choline (a), betaine (b), and DMG (c). The collected specimens were analyzed to measure choline and its metabolites by LC/MS/MS. d–f Characterization of plasma VLDL. The collected specimens were analyzed to evaluate the content of TG (d) and cholesterol (e) in VLDL and particle size (f) of VLDL using gel filtration high-performance liquid chromatography. Box-and-whisker plots are provided; the central line denotes the median value, the edges represent the upper and lower quartiles, and the whiskers indicate the minimum and maximum values. P values were calculated by Welch’s one-way ANOVA with a post hoc Dunnett’s T3 test for multiple comparisons and are indicated in the figures if less than 0.05. DMG dimethylglycine.
