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The role of ELOVL1 in very long-chain fatty acid homeostasis and X-linked adrenoleukodystrophy

X-linked adrenoleukodystrophy (X-ALD) is caused by mutations in the ABCD1 gene encoding the peroxisomal ABC transporter adrenoleukodystrophy protein (ALDP). X-ALD is characterized by the accumulation of very long-chain fatty acids (VLCFA; ≥C24) in plasma and tissues. In this manuscript we provide in...

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Detalles Bibliográficos
Autores principales: Ofman, Rob, Dijkstra, Inge M E, van Roermund, Carlo W T, Burger, Nena, Turkenburg, Marjolein, van Cruchten, Arno, van Engen, Catherine E, Wanders, Ronald J A, Kemp, Stephan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: WILEY-VCH Verlag 2010
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3377275/
https://www.ncbi.nlm.nih.gov/pubmed/20166112
http://dx.doi.org/10.1002/emmm.201000061