Washington: A team of scientists has revealed that Hutchinson-Gilford Progeria Syndrome (HGPS) related defects can be reduced with the help of a substance found in broccoli.
Children who suffer from the disease age prematurely due to a defective protein in their cells, but scientists at Technische Universitat Munchen (TUM) have now identified another important pathological factor, like, the system responsible for removing cellular debris and for breaking down defective proteins operates at lower levels in HGPS cells than in normal cells.
Most HGPS patients carry a mutation that produces a defective form of the protein lamin A, which is referred to as progerin and the normal lamin A is a key component of the matrix surrounding the DNA in the cell nucleus and plays a role in gene expression.
By contrast, the defective form, progerin, is not functional but is nevertheless continuously synthesized. The result is that progerin accumulates in the nucleus and causes the cell to "age". Consequently, HGPS patients develop classic diseases of old age such as atherosclerosis, osteoporosis, heart attacks and strokes. The disease is therefore regarded as a possible model system for the natural aging process in cells.
Researcher Karima Djabali added that progerin is also produced in healthy cells, probably as a byproduct and a well-functioning cellular waste disposal system can break down these small quantities of progerin. The scientists found, however, 10 to 20 times more progerin in the nuclei of diseased cells, a huge backlog of debris that needs to be removed.
However, scientists found a substance in broccoli called sulforaphane that activates protein degradation in cells and then treated the HGPS cells with the substance and found that significantly less progerin accumulated within the cells. Moreover, DNA damage and nuclear deformations, other effects of the disease, were also reduced in treated cells as compared with untreated cells.
Djabali explained that of course their experiments are very basic, but every active substance and every new approach brings us a step closer to a treatment for HGPS patients. It could also help us develop anti-aging strategies in the future.