Article
Diminished Canonical β-Catenin Signaling During Osteoblast Differentiation Contributes to Osteopenia in Progeria.
Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research - 1 Nov 2018
Choi Ji Young, Lai Jim K, Xiong Zheng-Mei, Ren Margaret, Moorer Megan C, Stains Joseph P, Cao Kan
Abstract excerpt
Patients with Hutchinson-Gilford progeria syndrome (HGPS) have low bone mass and an atypical skeletal geometry that manifests in a high risk of fractures. Using both in vitro and in vivo models of HGPS, we demonstrate that defects in the canonical WNT/β-catenin pathway, seemingly at the level of the efficiency of nuclear import of β-catenin, impair osteoblast differentiation and that restoring β-catenin activity...
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