Figure 1

Defects in Fatp4−/− lamellar body secretory system ultrastructure. Lamellar body structure ((a–d), reduced osmium tetroxide post-fixation) and extracellular lipid processing ((e–h), ruthenium tetroxide post-fixation) were evaluated by transmission electron microscopy. Typical abnormal curved membrane structures, seen only in Fatp4−/− epidermis, are shown in the inset in (b). Fatp4−/− lamellar bodies (b) were irregularly shaped and incompletely filled (black arrows) compared to control (a) and Fatp4−/− rescued with FATP4 (c) or FATP1 (d) (white arrows show normal lamellar bodies). Secreted lipid in Fatp4−/− was reduced in quantity, as seen by thin sheets of lamellar lipids ((f), black arrows), and exhibited extensive phase separation (white boxes) compared to control (e) and Fatp4−/− rescued with FATP4 (g) or FATP1 (h) (white arrows show normally processed lamellar lipids). Representative images are shown from two mice of each genotype.