Abstract
Peroxisomal disorders are genetically heterogeneous metabolic disorders associated with a deficit of very long chain fatty acid β-oxidation that commonly manifest as early-onset neurodegeneration. Brain microvascular endothelial dysfunction with increased permeability to monocytes has been described in X-linked adrenoleukodystrophy, one of the most common peroxisomal disorders caused by mutations of the ATP binding cassette subfamily D member 1 (ABCD1) gene. The present study demonstrated that dysregulation of sirtuin 1 (SIRT1) in human brain microvascular endothelial cells (HBMECs) mediates changes in adhesion molecules and tight-junction protein expression, as well as increased adhesion to monocytes associated with peroxisomal dysfunction due to ABCD1 or hydroxysteroid 17-β dehydrogenase 4 silencing. Furthermore, enhancement of the function of SIRT1 by resve-ratrol attenuated this molecular and functional dysregulation of HBMECs via modulation of the nuclear factor-κB and Krüppel-like factor 4 signaling pathways.
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