Researchers from the National Human Genome Research Institute, NIH, Bethesda, Maryland, and the Department of Cell Biology and Molecular Genetics, University of Maryland in the USA have discovered a previously unknown link between Progeria and aging. Their findings provide insights about the relationship between the toxic, Progeria-causing protein known as progerin and telomeres, which protect the ends of DNA within cells until they wear away over time and the cells die.
For the first time, we know that telomere shortening and dysfunction influences the production of progerin," says doctor Leslie B. Gordon, medical director of the Progeria Research Foundation. "Thus these two processes, both of which influence cellular aging, are actually linked."Prior research has shown that progerin is not only produced in children with Progeria, but that it is produced in smaller amounts in all of us, and progerin levels increase with aging. Independently, previous research on telomere shortening and dysfunction has been associated with normal aging. Since 2003, with the discovery of the Progeria gene mutation and the progerin protein that causes the disease, one of the key areas of research has focused on understanding whether and how Progeria and aging are linked.