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. 2021 Mar 25;49(7):3692–3708. doi: 10.1093/nar/gkab194

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© The Author(s) 2021. Published by Oxford University Press on behalf of Nucleic Acids Research.

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.

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Figure 5. — Effect of CC-885 and CC-90009 on IDUA PTC readthrough. (A, B) Fibroblasts derived from an unaffected individual were exposed to various concentrations of CC-885 (A) or CC-90009 (B) without or with 20 μg/ml G418 for 48 h and cell viability was measured using the MTT assay in triplicate samples (±SD, n = 3). (C, D) GM00798 primary fibroblasts derived from a MPS I-H patient with IDUA nonsense mutations (W402X/W402X) were exposed to the indicated concentrations of CC-885 (C) or CC-90009 (D) for 72 h and eRF3a, eRF3b and eRF1 levels were measured. GAPDH was used as loading control. (E, F) GM00798 fibroblasts were exposed to the same concentrations of CC-885 (E) and CC-90009 (F) as in C and D, with or without 20 μg/ml G418 for 72 h and α-l-iduronidase activity was measured in triplicate samples (±SD) and plotted as % of the activity measured in WT fibroblasts. * indicates statistically significant difference to untreated samples (P < 0.01).