Journal
AGING CELL
Volume 10, Issue 6, Pages 1011-1020Publisher
WILEY-BLACKWELL
DOI: 10.1111/j.1474-9726.2011.00743.x
Keywords
Hutchinson-Gilford progeria syndrome; premature aging; LMNA; laminopathy; nuclear lamina; stem cells
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Funding
- Swedish Medical Research Council
- Center for Biosciences
- Karolinska Institutet
- Marcus Borgstrom foundation
- Lars Hierta foundation
- Ake Wiberg foundation
- Svenska lakare sallskapet
- Karolinska Institutet KID
- Royal Swedish Academy of Sciences
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HutchinsonGilford progeria syndrome (HGPS or progeria) is a very rare genetic disorder with clinical features suggestive of premature aging. Here, we show that induced expression of the most common HGPS mutation (LMNA c.1824C>T, p.G608G) results in a decreased epidermal population of adult stem cells and impaired wound healing in mice. Isolation and growth of primary keratinocytes from these mice demonstrated a reduced proliferative potential and ability to form colonies. Downregulation of the epidermal stem cell maintenance protein p63 with accompanying activation of DNA repair and premature senescence was the probable cause of this loss of adult stem cells. Additionally, upregulation of multiple genes in major inflammatory pathways indicated an activated inflammatory response. This response has also been associated with normal aging, emphasizing the importance of studying progeria to increase the understanding of the normal aging process.
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