Metabolic rerouting via SCD1 induction impacts X-linked adrenoleukodystrophy

X-linked adrenoleukodystrophy (ALD) is a progressive neurodegenerative disease caused by mutations in ABCD1, the peroxisomal very long–chain fatty acid (VLCFA) transporter. ABCD1 deficiency results in accumulation of saturated VLCFAs. A drug screen using a phenotypic motor assay in a zebrafish ALD m...

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Detalhes bibliográficos
Publicado no:J Clin Invest
Main Authors: Raas, Quentin, van de Beek, Malu-Clair, Forss-Petter, Sonja, Dijkstra, Inge M.E., Deschiffart, Abigail, Freshner, Briana C., Stevenson, Tamara J., Jaspers, Yorrick R.J., Nagtzaam, Liselotte, Wanders, Ronald J.A., van Weeghel, Michel, Engelen-Lee, Joo-Yeon, Engelen, Marc, Eichler, Florian, Berger, Johannes, Bonkowsky, Joshua L., Kemp, Stephan
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Clinical Investigation 2021
Assuntos:
Research Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC8262477/
https://ncbi.nlm.nih.gov/pubmed/33690217
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1172/JCI142500
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