Supplemental Figure S1.
Fatty acid (FA) and ceramide synthetic pathway in autosomal recessive congenital ichthyosis converge on the corneocyte lipid envelope (CLE). A: Lipid metabolic pathway leading to formation of the CLE. Note close association of ichthyin and fatty acid transporter 4 (FATP4) in this pathway. B: Structure of the CLE and association to underlying cornified envelope (CE) (purple arrows). acylCer, ω-O-acylceramide; β-GlcCer'ase; β-glucocerebrosidase; FFA, free fatty acid; Glc, glucose; ω-acylGlcCer, ω-acyl-glucosylceramide; ω-OH-Cer, ω-hydroxyceramide; ω-OH-FA-CoA, ω-hydroxy-fatty acid-coenzyme A; ω-OH-FFA, ω-hydroxy-free fatty acid. Modified from Elias et al,5 with permission from Elsevier.