Experimental Studies on Werner’s Syndrome Fibroblasts

Nucleolar localization of the Werner syndrome protein in human cells

“…Cells explanted from individuals with WS show a greatly shortened division potential in vitro (3)(4)(5). Genomic instability has been observed in WS cells, including chromosomal rearrangements (6), as well as an increased frequency of deletion mutations within the HPRT gene (7).…”

The gene responsible for Werner syndrome may be a cell division "counting" gene.

“…Finally, the relevance of in vitro growth of human cell cultures to human aging is graphically demonstrated by Werner syndrome, which clearly mimics certain aspects of human aging (6). Fibroblast cultures from Werner syndrome patients show a dramatic shortening ofthe life-span in culture (13)(14)(15)(16). Normal human fibroblasts achieve %60 population doublings in culture, while Werner syndrome cells usually only achieve =20 population doublings.…”