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The syndrome is most often caused by mutations in the RECQL4 helicase, a member of the RecQ helicase family, which is involved in DNA repair and replication (Croteau et al

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Capsules:30

Nrf1/2 maintains mitochondrial homeostasis by modulating the nuclear gene expression of components within the oxidative phosphorylation system (Scarpulla, 2002a,b)