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Expression of the Hutchinson-Gilford Progeria Mutation during Osteoblast Development Results in Loss of Osteocytes, Irregular Mineralization, and Poor Biomechanical Properties

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...

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Autors principals: Schmidt, Eva, Nilsson, Ola, Koskela, Antti, Tuukkanen, Juha, Ohlsson, Claes, Rozell, Björn, Eriksson, Maria
Format: Artigo
Idioma:Inglês
Publicat: American Society for Biochemistry and Molecular Biology 2012
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Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC3460452/
https://ncbi.nlm.nih.gov/pubmed/22893709
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M112.366450
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