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Targeted metabolomic analyses of cellular models of pelizaeus-merzbacher disease reveal plasmalogen and myo-inositol solute carrier dysfunction

BACKGROUND: Leukodystrophies are devastating diseases characterized by dys- and hypo-myelination. While there are a number of histological and imaging studies of these disorders, there are limited biochemical data available. We undertook targeted lipidomic analyses of Pelizaeus-Merzbacher disease (P...

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Detalles Bibliográficos
Autores principales: Wood, Paul L, Smith, Tara, Pelzer, Lindsay, Goodenowe, Dayan B
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3141545/
https://www.ncbi.nlm.nih.gov/pubmed/21682894
http://dx.doi.org/10.1186/1476-511X-10-102