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

BACKGROUND: Childhood peroxisomal disorders and leukodystrophies are devastating diseases characterized by dysfunctional lipid metabolism. Plasmalogens (ether glycerophosphoethanolamine lipids) are decreased in these genetic disorders. The biosynthesis of plasmalogens is initiated in peroxisomes but...

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Detalles Bibliográficos
Autores principales: Wood, Paul L, Khan, M Amin, Smith, Tara, Ehrmantraut, Greg, Jin, Wei, Cui, Wei, Braverman, Nancy E, Goodenowe, Dayan B
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2011
Materias:
Research
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3238230/
https://www.ncbi.nlm.nih.gov/pubmed/22008564
http://dx.doi.org/10.1186/1476-511X-10-182

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