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Xueping Chen, Xiaosha Zhang, Chen Li, Teng Guan, Huifang Shang, Liying Cui, Xin-Min Li and Jiming Kong S-nitrosylated protein disulfide isomerase contributes to mutant SOD1 aggregates in amyotrophic lateral sclerosis Journal of Neurochemistry 124

Version of Record online: 1 NOV 2012 | DOI: 10.1111/jnc.12046

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We proposed a role of nitrosative stress in the formation of SOD1 aggregates in transgenic mice expressing mutant SOD1: inducible nitric oxide synthase up-regulation generates high levels of nitric oxide and subsequently induces S-nitrosylation of PDI, leading to SOD1 misfolding and aggregation. Our findings suggest that PDI should be a target for new therapeutic strategies for ALS.

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