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. 2008 Apr 7;181(1):9–13. doi: 10.1083/jcb.200802155

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Copyright © 2008, The Rockefeller University Press

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Figure 2. — A model for mesenchymal stem cell dysfunction in HGPS. In wild-type mesenchymal stem cells (MSC; left), Notch signaling, which operates through the cleavable and nuclear-penetrating Notch intracellular domain (NICD), is active at basal levels, resulting in basal expression of Notch downstream targets, including Hes1, Hes3, Hes5, Hey1, Hey2, and TLE1. In Hutchison-Gilford progeria syndrome (HGPS), Notch signaling is increased in mesenchymal stem cells (right), leading to an increase in the expression of Notch pathway genes and to perturbed differentiation, including increased osteogenesis and decreased adipogenesis. INM, inner nuclear membrane.