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X-linked adrenoleukodystrophy: very long-chain fatty acid metabolism is severely impaired in monocytes but not in lymphocytes
X-linked adrenoleukodystrophy (X-ALD) is a fatal neurodegenerative disease caused by mutations in the ABCD1 gene, encoding a member of the peroxisomal ABC transporter family. The ABCD1 protein transports CoA-activated very long-chain fatty acids (VLCFAs) into peroxisomes for degradation via β-oxidat...
Autores principales: | Weber, Franziska D., Wiesinger, Christoph, Forss-Petter, Sonja, Regelsberger, Günther, Einwich, Angelika, Weber, Willi H.A., Köhler, Wolfgang, Stockinger, Hannes, Berger, Johannes |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3990157/ https://www.ncbi.nlm.nih.gov/pubmed/24363066 http://dx.doi.org/10.1093/hmg/ddt645 |
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