Progeria is an extremely rare disease where children age prematurely. Progerin, a protein, acts by deforming the nuclei of cells in children characterized by nuclei in cells of children that are poor at breaking down and disposing of defective proteins.
Progerin accumulates and interferes with a number of cell functions. It is also found in healthy cells but at a much lower level.
Patients affected by progeria posses a gene variant that produces a faulty version of a protein called lamin A. Normal lamin A is an important component of the matrix that surrounds DNA in the cell nucleus and plays a key role in gene expression. The progerin variant of lamin A does not have a function; it just becomes a nuisance as it accumulates because the cell continues to make it as instructed by the faulty gene.
However, as it collects in the nucleus, progerin causes the cell to “age,” meaning patients with the disease develop classic health problems of old age, such as atherosclerosis, osteoporosis, stroke and heart attack.
Researchers from the Technical University at Munich (TUM) have described how they succeeded in reducing levels of progerin in diseased cells by reactivating a protein-clearing process.
Sulforaphane is a potent antioxidant found in broccoli and other cruciferous vegetables, such as cabbage and Brussels sprouts and acts by reactivating protein and debris elimination in the cell nuclei, thus combating the impact of progerin accumulation. Progeria diseased cells treated with sulforaphane also appeared to have less DNA damage and nuclear deformations.
Sulforaphane enhances progerin clearance in Hutchinson–Gilford progeria fibroblastst, Diana Gabriel, et al., Aging Cell, doi:10.1111/acel.12300, published online 16 December 2014.