<it>In vitro </it>and <it>in vivo </it>plasmalogen replacement evaluations in rhizomelic chrondrodysplasia punctata and Pelizaeus-Merzbacher disease using PPI-1011, an ether lipid plasmalogen precursor

<it>In vitro </it>and <it>in vivo </it>plasmalogen replacement evaluations in rhizomelic chrondrodysplasia punctata and Pelizaeus-Merzbacher disease using PPI-1011, an ether lipid plasmalogen precursor

<p>Abstract</p> <p>Background</p> <p>Childhood peroxisomal disorders and leukodystrophies are devastating diseases characterized by dysfunctional lipid metabolism. Plasmalogens (ether glycerophosphoethanolamine lipids) are decreased in these genetic disorders. The biosy...

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Bibliographic Details
Main Authors: Wood Paul L, Khan M Amin, Smith Tara, Ehrmantraut Greg, Jin Wei, Cui Wei, Braverman Nancy E, Goodenowe Dayan B
Format: Article
Language:English
Published: BMC 2011-10-01
Series:Lipids in Health and Disease
Subjects:
Rhizomelic chrondrodysplasia punctata type 1
Rhizomelic chrondrodysplasia punctata type 2
Pelizaeus-Merzbacher disease
Pex7 mouse
lymphocytes
plasmalogen precursor
DHA
peroxisomal disorders
PPI-1011
Online Access:http://www.lipidworld.com/content/10/1/182

Internet

http://www.lipidworld.com/content/10/1/182

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