Blood Vessels Developed to Screen Progeria

Hutchison-Gilford Progeria Syndrome (HGPS) is an extremely rare genetic disorder where the affected person shows accelerated aging - a young child appears like an old person. The lifespan of these individuals is short - usually less than 14 years. Affected individuals usually die of cardiovascular disease. Progeria results due to the accumulation of a protein called progerin within cells. Changes noted in the smooth muscle layer of blood vessels, which is the middle layer of the blood vessels, include excess calcium and fat deposits, thickening of the blood vessel layer and fibrosis. Scientists have developed blood vessels in the laboratory that are similar to the blood vessels of progeria patients. These can serve as a model to screen drugs that could possibly be useful in patients with the rare genetic disorder.