Journal: Lipids in Health and Disease
Article Title: In vitro and in vivo plasmalogen replacement evaluations in rhizomelic chrondrodysplasia punctata and Pelizaeus-Merzbacher disease using PPI-1011, an ether lipid plasmalogen precursor
doi: 10.1186/1476-511X-10-182
Figure Lengend Snippet: Ethanolamine plasmalogen levels in RCDP1 and RCDP2 lymphocytes with 0, 20 or 100 μM PPI-1011 for 72 hr . N = 8. Mean ± SEM. All basal decrements and PPI-1011-dependent increases were significantly (p < 0.01) from control lymphocytes and from 0 μM PPI-1011, respectively. 16:0 (palmitic acid), 18:1 (oleic acid), 18:2 (linoleic acid), 20:4 (arachidonic acid), 22:6 (docosahexaenoic acid; DHA).
Article Snippet: Control lymphocytes (Coriell GM13072 and GM02184); PMD lymphocytes (Coriell GM09545; PLP1 c.767C > T or p.P215S); RCDP1 lymphocytes (Coriell GM09291; PEX7 c.870 871insCAA/875T > A or p.C290 E291insQ/L292X and show a severe plasmalogen synthesis defect) and RCDP2 lymphocytes (Coriell GM16776; homozygous for GNPT c.1280-3T > G predicted to encode an in-frame protein p.427 507 del and showing a milder plasmalogen synthesis defect) [ ] were kept as suspension cultures (25 ml flasks) in RPMI 1640 (Hyclone) supplemented with 10% FBS and 1% antibiotic/antimycotic, at 37°C in a 5% CO 2 incubator [ ].
Techniques: