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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