Characterization of IPSC-derived brain cells from AMN and cALD for metabolic defect.

(A-B) Quantification of VLCFA (C26:0 μg/mg of protein or ratio C26:0/C22:0) in neurons, OLs and Ast from different cell lines (Control, AMN and cALD) by GC. In brief, fatty acids methyl ester was prepared directly from different cell types as described in Material and Methods. (A) VLCFA (C26:0 and C22:0) were measured as area percent of total FAs and expressed as ratio of C26:0/22:0 or (B) normalized to protein and presented as absolute amount per mg of protein in Control, AMN and cALD OLs and Ast. Results represent the means±SD from three different cell differentiation experiments; *PELOVL1 in different IPSC-derived brain cells from Control, AMN and cALD by RT-qPCR (n = 3); n is the number of independent preparation of cells. mRNA levels were standardized with mRNA level of the GAPDH or RPLP0. Data are represented as mean±SD. *P