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As reported in Figure 2, while the GCL expression levels in I-1, II-2, and proband II-1 were comparable with those of the controls (Figures 2A,B), the asymptomatic mother (I-2) showed a significant upregulation of the GCL gene, either in leukocytes (Figure 2A) or in fibroblasts (Figure 2B), probably as a response to the low availability of GSH (Figures 1B,D)

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