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Preventing Progeria

May 28, 2013

Scientists have identified an enzyme that could be responsible for one of the rarest childhood diseases of all.

Transcript

BOB HIRSHON (host):

Preventing progeria. I’m Bob Hirshon and this is Science Update.

Children born with a rare condition called progeria show many signs of accelerated aging, including hair loss, wrinkled skin, bone defects, and high blood pressure. Most die from heart disease before age twenty. There is currently no treatment for progeria, but now, scientists have discovered that blocking an enzyme called ICMT can prevent the condition in mice. University of Gothenburg biologist Martin Bergö explains.

MARTIN BERGÖ (University of Gothenburg):

When we reduced the activity of ICMT, this prevented the appearance of all of the classical progeria-like symptoms, and it prevented the death of the mice.

HIRSHON:

Bergö says progeria is caused by a genetic mutation that destabilizes the nucleus of the cell. Turning off ICMT restores the nucleus, allowing the cell to function properly. It may be several years before the ICMT-blocking drugs can be tested in humans. I’m Bob Hirshon, for AAAS, the science society. 

Two children with progeria. (Photo courtesy of The Progeria Research Foundation)