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:

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: Incorporation of PPI-1038 (100 μM; 72 hr) into the 16:0/22:6, 18:0/22:6 and 18:1/22:6 plasmalogens and DHA of RCDP1 and RCDP2 lymphocytes . N = 6. Mean ± SEM. P =[ 13 C 16 ]palmitic acid; G =[ 13 C 3 ]glycerol; D = [ 13 C 3 ]DHA. *, p < 0.01 vs. control.

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:

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 Pex7 mouse tissues (liver, kidney, heart, lung, neocortex and eye) . N = 5 Pex 7; N = 10 controls (2 heterozygotes + 8 wild-type). Mean ± SEM. All plasmalogen decreases were statistically significant (p < 0.05).

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:

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: Incorporation of [ 13 C 16 ]palmitic acid and [ 13 C 3 ]glycerol from PPI-1038 (100 mg/kg/day for 3 days) into Pex7 tissue PlsEtn 16:0/22:6 . N = 4. Mean ± SD. Increases in labeling in the Pex7 mice were significant in all cases (p < 0.01). HT, heterozygote controls; HO, homozygotes.

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: Labeling

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: Incorporation of [ 13 C 6 ]palmitic acid and [ 13 C 3 ]glycerol (*) from PPI-1038 (100 mg/kg/day for 3 days) into PlsEtn 16:0/22:6 in the neocortex and eyes of Pex7 mice . Similarly the incorporation of [ 13 C 3 ]DHA (**) into PlsEtn 16:0/22:6, PlsEtn 18:0/22:6 and PlsEtn 18:1/22:6 is presented. N = 4. Mean ± SD. Increases in labeling in the Pex7 mice were significant in all cases (p < 0.01). HT, heterozygote controls; HO, homozygotes. * = [ 13 C 16 ]palmitic acid + [ 13 C 3 ]glycerol labeled PlsEtn x/22:6; ** = [ 13 C 3 ]DHA labeled PlsEn x/22:6.

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: Labeling