Patients with Hutchinson-Gilford Progeria Syndrome age eight to 10 times faster than the rest of us and rarely live beyond 13 years. Almost all of the patients die from complications of arteriosclerosis, the clogging or hardening of arteries or blood vessels caused by plaques, which leads to heart attack and stroke.
Research on Progeria is difficult because the disease is exceedingly rare and only 64 children living with progeria are known, making access to patients very difficult.
Now, researchers at the Salk Institute for Biological Studies have replicated premature aging in the lab, allowing them to study aging-related disease in a dish. Writing in Nature, they report that they have successfully generated induced pluripotent stem (iPS) cells from skin cells obtained from patients with Hutchinson-Gilford Progeria Syndrome—who age eight to 10 times faster than the rest of us—and differentiated them into smooth muscle cells displaying the telltale signs of vascular aging.
"There is also evidence that defective lamin A accumulates during the normal aging process via the sporadic use of the alternative splice site," explains Juan Carlos Izpisúa Belmonte, Ph.D. a professor in the Salk Institute's Gene Expression Laboratory. "Therefore we are very keen on using our in vitro iPS cell-based model to identify new aging markers and explore other aspects of human premature and physiological aging."