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Cells from these patients display increased oxidative stress as a consequence of elevated levels of ROS, increased oxidized/reduced glutathione (GSSG/GSH) ratio, diminished capacity to scavenge ROS and mitochondrial dysfunction.1812 Furthermore, ATM is directly activated by oxidative stress19 and can exert antioxidant activity through regulation of the pentose-phosphate pathway.20 Recent evidence suggests that ATM might regulate oxidative stress through NRF2

Treatment : Transfusions: Hb values fall below 7gr / dl Facial deformations Poor growth Bone expansion Increased splenomegaly Treatment of Fe overload Splenectomy Chelation therapy: Administer Iron chelators for elimination in urine and feces (from 10 to 20 transfusions or at ferritin levels above 1000 ng/ml)

Nrf2 activation protects individuals against cerebral ischemic damage

Mitochondrial protein import stress augments -synuclein aggregation and neurodegeneration independent of bioenergetics

(2018), along with other complications