Hutchinson-Gilford progeria syndrome (HGPS, OMIM 176670) is a rare disorder 
characterized by segmental accelerated aging and early death from coronary 
artery disease or stroke. Nearly 90% of HGPS sufferers carry a G608G mutation 
within exon 11 of LMNA, producing a truncated form of prelamin A, referred to as 
"progerin". Here, we report the isolation of naïve multipotent skin-derived 
precursor (SKP) cells from dermal fibroblast cultures from HGPS donors. These 
cells form spheres and express the neural crest marker, nestin, in addition to 
the multipotent markers, OCT4, Sox2, Nanog and TG30; these cells can self-renew 
and differentiate into smooth muscle cells (SMCs) and fibroblasts. The SMCs 
derived from the HGPS-SKPs accumulate nuclear progerin with increasing passages. 
A subset of the HGPS-naïve SKPs express progerin in vitro and in situ in HGPS 
skin sections. This is the first in vivo evidence that progerin is produced in 
adult stem cells, and implies that this protein could induce stem cells 
exhaustion as a mechanism contributing to aging. Our study provides a basis on 
which to explore therapeutic applications for HGPS stem cells and opens avenues 
for investigating the pathogenesis of other genetic diseases.