Is Telomere Shortening Related to Progeria?

Abstract

Editor's Note: Hutchinson-Gilford progeria is a segmental progeria of early childhood onset. One of the questions that has intrigued many of us over the years has been the extent to which cell senescence (or telomere length, as a poor person's marker marker for cell senescence) might play a role in this disease in which the children typically die of what we normally consider to be age-related diseases (generally atherosclerotic complications). Several lines of evidence suggest that this might be the case, but the role of cell senescence, if any, remains unclear. One current project involves the possibility of using gene transfection to settle the issue directly. In the meantime, what data we have is insufficient. Ted Brown and I are two of the three physicians (Frank DeBusk is the other) who see these children annually as they meet from all over the world. We know them all. I invited Dr. Brown to discuss his opinion on the role of cell senescence in progeria because: (1) I disagree with him; (2) he knows more about the clinical features of these children than I do; and (3) I respect his opinion and his thinking.