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Pharmacological complementation remedies an inborn error of lipid metabolism

X-linked adrenoleukodystrophy (X-ALD) is a rare, genetic disease in which increased very long chain fatty acids (VLCFAs) in the central nervous system (CNS) cause demyelination and axonopathy, leading to neurological deficits. Sobetirome, a potent thyroid hormone agonist, has been shown to lower VLC...

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Détails bibliographiques
Publié dans:Cell Chem Biol
Auteurs principaux: Hartley, Meredith D., Shokat, Mitra D., DeBell, Margaret J., Banerji, Tania, Kirkemo, Lisa L., Scanlan, Thomas S.
Format: Artigo
Langue:Inglês
Publié: 2020
Sujets:
Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC7245556/
https://ncbi.nlm.nih.gov/pubmed/32169163
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.chembiol.2020.02.008
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