[HTML][HTML] Expression of the Hutchinson-Gilford progeria mutation during osteoblast development results in loss of osteocytes, irregular mineralization, and poor …
E Schmidt, O Nilsson, A Koskela, J Tuukkanen… - Journal of biological …, 2012 - ASBMB
Hutchinson-Gilford progeria syndrome (HGPS) is a very rare genetic disorder that is
characterized by multiple features of premature aging and largely affects tissues of
mesenchymal origin. In this study, we describe the development of a tissue-specific mouse
model that overexpresses the most common HGPS mutation (LMNA, c. 1824C> T, p.
G608G) in osteoblasts. Already at the age of 5 weeks, HGPS mutant mice show growth
retardation, imbalanced gait and spontaneous fractures. Histopathological examination …
characterized by multiple features of premature aging and largely affects tissues of
mesenchymal origin. In this study, we describe the development of a tissue-specific mouse
model that overexpresses the most common HGPS mutation (LMNA, c. 1824C> T, p.
G608G) in osteoblasts. Already at the age of 5 weeks, HGPS mutant mice show growth
retardation, imbalanced gait and spontaneous fractures. Histopathological examination …