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Figure 7 | Lipids in Health and Disease

Figure 7

From: In vitro and in vivo plasmalogen replacement evaluations in rhizomelic chrondrodysplasia punctata and Pelizaeus-Merzbacher disease using PPI-1011, an ether lipid plasmalogen precursor

Figure 7

Conversion 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].

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