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article imageBroccoli can help counter a rare genetic disease

By Tim Sandle     Dec 20, 2014 in Science
A low number of children around the world suffer with a rare disease called Hutchinson-Gilford Progeria syndrome. This condition leads to premature ageing and it is caused by a defective protein. A substance from broccoli may provide a treatment.
Hutchinson-Gilford Progeria syndrome (HGPS) is an extremely rare genetic disorder wherein symptoms resembling aspects of aging are manifested at a very early age. Those born with progeria typically live to their mid-teens to early twenties. The disease is genetic and it normally occurs through a mutation, rather than being inherited.
HGPS patients carry a mutation that produces a defective form of a protein called lamin A (or progerin, in the defective form.) Lamin A is a necessary for the cell nucleus and plays a key role in how genes express (which means how the physical features develop.) When in the defective form, progerin continuous to synthesize and it accumulates in the cell. This triggers the cell to "age."
With the disease, scientists have identified the system responsible for breaking down defective proteins in HGPS cells, which is different to normal cells. A science group have succeeded in reactivating protein breakdown affected cells. This has lowered the disease-related defects. This has come about by using a substance extracted from broccoli.
The substance in broccoli called sulforaphane. This substance appears to activate protein degradation in cells, which decreases progeria and limits the ageing effect. Sulforaphane is obtained from cruciferous vegetables such as broccoli, Brussels sprouts or cabbages. Sulforaphane has attracted recent research attention for its antioxidant properties, which may persist for hours after ingestion.
The research was conducted at Technische Universität München (TUM). The findings have been published in the journal Aging Cell, in a paper headed “Sulforaphane enhances progerin clearance in Hutchinson-Gilford progeria fibroblasts.”
More about Rare disease, Genes, HutchinsonGilford Progeria Syndrome, progeria
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