Figure 7From: In vitro and in vivo plasmalogen replacement evaluations in rhizomelic chrondrodysplasia punctata and Pelizaeus-Merzbacher disease using PPI-1011, an ether lipid plasmalogen precursorConversion of PPI-1011 to the target plasmalogen PlsEtn 16:0/22:6. Removal of the sn-3 lipoic acid by lipases is followed in the endoplasmic reticulum by addition of phosphoethanolamine to the glycerol backbone at sn-3 (EC 3.1.3.4) and desaturation of the ether linked fatty acid at sn-1 (EC 1.14.99.19). Subsequent lipid remodeling at sn-2 is mediated by tightly coupled deacylation/reacylation enzyme reactions [10].Back to article page