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A-T patients lack fully functioning ATM and are predisposed to a range of malignancies, radiation sensitivity, immunological and neurological disorders 56 caused by abnormalities in the cells response to DNA repair and elevated ROS levels particularly in neurons 57

Usp2 OE also accentuated UUO-induced iron accumulation and disrupted redox homeostasis, as evidenced by increased MDA levels, reduced SOD activity, and a diminished GSH/GSSG ratio (Fig

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