2-Methylbutyrylglycine induces lipid oxidative damage and decreases the antioxidant defenses in rat brain

► 2MBG and 2MB accumulate in SBCAD deficiency. ► 2MBG induces brain lipid oxidative damage. ► 2MBG reduces nonenzymatic brain antioxidant defenses. ► 2MBG-induced effects are possibly mediated by reactive oxygen species. ► Oxidative stress may contribute to the neuropathology found in SBCAD deficiency.