Impaired membrane traffic in defective ether lipid biosynthesis

The first steps of ether lipid biosynthesis are exclusively localized to pe roxisomes and hence some peroxisomal disorders are characterized by a sever e deficiency of plasmalogens, the main ether lipids in humans. Here we repo rt on gene defects of plasmalogen biosynthesis, chromosomal localization of the corresponding genes and, as a consequence of plasmalogen deficiency, o n structural alterations of caveolae, clathrin-coated pits, endoplasmic ret iculum and Golgi cisternae, as well as on the reduced rate of transferrin r eceptor cycling. The data suggest that plasmalogens, analogous to cholester ol, are essential for correct membrane functioning and their deficiency res ults in impaired membrane trafficking.