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Latest Mprize Winners [online], (2013) Bonkowski, M
Copper transporter deficiency in ATP7A-deficient Menkes disease-mimicking fibroblasts is associated with mitochondrial redox imbalance that contribute to toxicity, related to copper overload [3]
Abdulghani AA et al
Overall, whilst these findings imply that increased production of ROS is associated with C9orf72-ALS, it is unclear whether loss of C9orf72 or expression of hexanucleotide repeat expansions disturbs redox homeostasis
Although an expected reduction in vasopressor requirement was observed with exogenous Ang II administration in the ATHOS-3 trial [28], some patients did not have this vasoconstrictive response, leading to the hypothesis that peptidases, such as DPP3, could be involved in the lack of response to exogenous Ang II [12, 29]
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