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New hope for a rare illness causing accelerated ageing

24-07-2008

Spanish and French scientists may have open the doors for treatment on progeria, a rare disease that causes accelerated and premature ageing. The ESRF provided the team with an X-rays irradiator to study the response of the DNA damage repair of cells from progeria patients.

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The gene causing progeria was discovered five years ago by a team from the French national health and medical research institute (INSERM) in Marseille (INSERM Unit 910). It affects children from their birth and the average life expectancy reaches only 13 years of age. It affects one every 4-8 million children worldwide.

 
progeria
 

 Megan, 6 years old. Photo courtesy of The Progeria Research Foundation. It is forbidden to download this image.

Today, the same team that unveiled the gene responsible for this disease, together with a team from the University of Oviedo, has succeeded, in animal models, in decreasing the effects of the disease and in slowing down its development. Notably, the life expectancy for the treated mice went from 101 days on average to 179 days.

The work at the ESRF consisted in analysing the effects of X-rays in cells from progeroid patients. The medical beamline allowed the team to irradiate the cells with a 200 kV X-rays irradiator and, after that, to study the DNA damage repair using immunofluorescence technique. The research at the ESRF was carried out by the Radiobiology Group of the local INSERM team (INSERM Unit 836 - team 6 ), which has a long-term collaboration with the facility.

Reference:
Varela et al., Nature Medicine, July 2008.

More information about the discovery of the progeria gene:

Eriksson et al, Nature, Vol. 423, May 15, 2003.