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Striatal Neurodevelopment Is Dysregulated in Purine Metabolism Deficiency and Impacts DARPP-32, BDNF/TrkB Expression and Signaling: New Insights on the Molecular and Cellular Basis of Lesch-Nyhan Syndrome

Figure 4

Bcl11b and Ppp1r1b/DARPP-32 in human fibroblasts.

(A) Bcl11b expression in individual fibroblast samples from five control (normal) subjects (C1–C5) and from five patients with LNS (L1–L5). Δct values are normalized to control mRNA (GAPDH). (B) Normalized Bcl11b expression in controls (CTL) and LNS patients. (C) Ppp1r1b/DARPP-32 expression in individual fibroblast samples from five control (normal) subjects (C1–C5) and from five patients with LNS (L1–L5). Δct values are normalized to control mRNA (GAPDH). (D) Normalized Ppp1r1b/DARPP-32 expression in controls (CTL) and LNS patients (*P<0.05, t-test).

Figure 4

doi: https://doi.org/10.1371/journal.pone.0096575.g004