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Nucleic Acids 21, 217228

When mitochondrial function is compromised by oxidative stress, cellular processes slow down, leading to decreased collagen production, impaired barrier function, and reduced skin hydration

81 , 119 (1959) Meldrum, N.U.: The reduction of glutathione in mammalian erythrocytes

While the molecular pathogenesis of AxD remains incompletely understood, massive reactive astrogliosis with Rosenthal fibers composed of aggregated GFAP, vimentin, nestin, B-crystallin and small heatshock proteins together with prominent leukodystrophy are the pathological hallmarks, with the pathophysiological picture including mitochondrial malfunction, redox imbalance and susceptibility to oxidative stress 331,332,333