By IANS
Washington : Adult stem cells may help explain what causes a rare disease that triggers premature aging in children, a new study has found.
These findings, the first to indicate a biological basis for the clinical features of what is known as the Hutchinson-Gilford Progeria Syndrome (HGPS) – known popularly as progeria – may provide insights into the biological mechanisms of normal aging.
The findings of the study have been published in the latest issue of the journal Nature Cell Biology.
HGPS is an extremely rare hereditary genetic disease that afflicts children. So far, only 100 patients have been documented in medical literature.
Those afflicted generally experience the first symptoms by the age of one, and on average succumb around the age of 15, almost exclusively from premature, progressive heart disease.
“Studies like these of HGPS hold the potential to benefit children suffering this terrible illness and enlighten us as to the medical changes we all experience as we grow older,” said lead researcher John E. Niederhuber of the National Cancer Institute (NCI).
Because its striking cardiovascular effects and other clinical features are so closely associated with the normal aging process, HGPS holds great interest for researchers studying age-related biological changes and disease.
The cause of HGPS, a mutated protein called progerin, was identified in 2003. However, the mechanism by which progerin causes the widespread clinical effects of HGPS has been unclear.
Tom Misteli and Paola Scaffidi of NCI looked at the effects of progerin on adult mesenchymal stem cells. An adult stem can renew itself, and can differentiate to yield major specialized cell types of the tissue or organ.
Their experiments revealed that progerin profoundly affects the fate of these stem cells, greatly skewing the rate at which they mature into different tissues.
For instance, progerin-producing stem cells showed accelerated maturation into bone but failed to develop into fat.
This could explain two of the distinguishing clinical features of HGPS: abnormal bone growth and an almost complete loss of the fatty tissues normally found just beneath the skin.