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Model of pathological mechanism resulting from expansion of first poly-A tract in Arx. The expansion results in a context-dependent loss of Tle recruitment to promoter sites. The context at the Ebf3 and Shox2 promoters does not allow Tle recruitment with ArxE (top right); however, the binding and activity of Tle are not perturbed in the case of Lmo1 (bottom right).
