Progress in Progeria Research
The accelerated aging condition progeria so far appears to be an extreme example of one type of cellular damage that usually only provides a very modest contribution to degenerative aging. Here is news of recent research: "Hutchinson-Gilford Progeria Syndrome, also known as progeria, is caused by a mutation in the gene encoding for the protein lamin A, an important component of the membrane surrounding a cell's nucleus. The mutation results in a truncated form of lamin A called progerin, which in turn causes misshapen cell nuclei and DNA damage. ... [researchers] have produced the world's first human cell model of progeria, a disease resulting in severe premature ageing in one in four to eight million children worldwide. This model has allowed them to make new discoveries concerning the mechanism by which progeria works. ... the team used a novel technique of deriving induced pluripotent stem (iPS) cells from cells of human progeria patients. This human progeria model allows the group to trace and analyse the distinctive characteristics of progeria as it progresses in human cells. ... The researchers used their iPS cells to identify two types of cells - mesenchymal stem cells (MSCs) and vascular smooth muscle cells (VSMCs) - that were particularly adversely affected by progeria. This means that a young patient with progeria would typically have fewer MSCs and VSMCs than other children. MSCs were found to be very sensitive to a low oxygen environment and their losses could delay renewal of the various tissues they gave rise to, thus exacerbating the patient's symptoms of ageing. The same effect on VSMCs could explain why their number was reduced in the patient's heart vessels. ... This new study provides further evidence for the role of lamin processing in connective tissue function, as well as insights into the normal ageing process. We hope to soon find new routes of intervention to treat this incurable disease. Such interventions may be of use in treating atherosclerosis in general, a condition afflicting many millions of individuals."
Link: http://www.sciencedaily.com/releases/2011/01/110124102948.htm