Scientists have discovered that they can dramatically increase the life span of mice with progeria (premature ageing disease) and heart disease (caused by Emery-Dreifuss muscular dystrophy) by reducing levels of a protein called SUN1. This research was done by A*STAR's Institute of Medical Biology (IMB) in collaboration with their partners at the National Institute of Allergy and Infectious Diseases in the United States and the Institute of Cellular and System Medicine in Taiwan. Their findings were published in the prestigious scientific journal, Cell, on 27th April 2012 and provide an exciting lead into developing new methods to treat premature aging and heart disease.
Children with progeria suffer symptoms of premature ageing and mostly die in their early teens from either heart attack or stroke. Individuals with Emery-Dreifuss muscular dystrophy (AD-EDMD) suffer from muscle wasting and cardiomyopathy, a type of heart disease that weakens and enlarges the heart muscle making it harder for the heart to pump blood and deliver it to the rest of the body leading to heart failure. Both diseases are caused by mutations in Lamin A, a protein in the membrane surrounding a cell's nucleus which provides mechanical support to the nucleus. SUN1 is a protein also found in the inner nuclear membrane, but there have been no previous studies to show how SUN1 interacts with the Lamin proteins.
The scientists wanted to investigate if SUN1 had any involvement in diseases caused by mutations in Lamin A, so they inactivated SUN1 in mouse models developed for progeria and AD-EDMD. These mouse models for progeria and AD-EDMD usually thrive poorly and have markedly short life spans as they die from premature ageing and heart failure respectively. However, by inactivating SUN1 and reducing SUN1 levels in these mouse models, the scientists observed that the life spans of the mouse models for progeria and AD-EDMD doubled and tripled respectively.
|Contact: Ong Siok Ming|
Agency for Science, Technology and Research (A*STAR), Singapore