Fig. 2.

PlsEtn levels of cultured RCDP fibroblasts correlate with disease severity. (A) Plasmalogen biosynthesis rates were determined by the method of Roscher et al. [51] and are depicted as ratio between the peroxisomal (¹⁴C) and the ER (³H) steps of biosynthesis. Typical biosynthesis rates of healthy controls are indicated by the shaded area (“reference range”). ***P ≤ 0.001; **P ≤ 0.01 (one-sample t-test versus reference range; two-tailed t-test comparing intermediate and severe RCDP fibroblasts) (B) PlsEtn levels were measured in primary human fibroblasts. PlsEtn subspecies are grouped according to their different sn-1 moieties indicated by 16:0, 18:0 and 18:1. Data are represented as means ± SD derived from at least three different cell lines per group. ***P ≤ 0.001; *P ≤ 0.05 (one-way ANOVA with Tukey's post hoc test for comparison of total plasmalogen levels).