21 research outputs found
Highâdose biotin restores redox balance, energy and lipid homeostasis, and axonal health in a model of adrenoleukodystrophy
No full textImpaired mitochondrial oxidative phosphorylation in the peroxisomal disease X-linked adrenoleukodystrophy
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25-hydroxycholesterol contributes to cerebral inflammation of X-linked adrenoleukodystrophy through activation of the NLRP3 inflammasome
No full textMitochondrial Bioenergetics Is Altered in Fibroblasts from Patients with Sporadic Alzheimer's Disease
No full textAutophagy induction halts axonal degeneration in a mouse model of X-adrenoleukodystrophy
No full textPeroxisomal membrane channel Pxmp2 in the mammary fat pad is essential for stromal lipid homeostasis and for development of mammary gland epithelium in mice
No full textTauroursodeoxycholic bile acid arrests axonal degeneration by inhibiting the unfolded protein response in X-linked adrenoleukodystrophy
No full textFunctional characterization of novel genotypes and cellular oxidative stress studies in propionic acidemia
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