Premature aging: scientists identify and correct defects in diseased cells

Scientists have succeeded in restoring normal activity in cells isolated from patients with the premature aging disease Cockayne syndrome. They have uncovered the role played in these cells by an enzyme, the HTRA3 protease. This enzyme is overexpressed in Cockayne syndrome patient cells, and leads to mitochondrial defects, which in turn play a crucial role in the appearance of symptoms leading to aging in affected children.