Common chemical reverses progeria symptoms and normal age-related damage
A new study by a team of scientists at the University of Maryland (UMD) indicates that a common chemical can reverse the symptoms of the premature-aging disease progeria and perhaps even those of normal aging. According to the study, small doses of methylene blue can undo the damage done to cells by the genetic defect that causes progeria with a speed and reliability that the scientists claim is "like magic."
Progeria is a rare, notorious, and tragic genetic disease that afflicts the young. The patients usually show symptoms in the first year of life, which are similar to normal aging, except greatly speeded up. They become thin, the bones and joints grow fragile, the skin wrinkles, all hair is lost, and death occurs from organ failure and complications by the time most reach their teens.
It's caused by a defect in the gene that controls the production of lamin A protein, which is part of the cellular nucleus under the nuclear membrane. According to the UMD team, healthy cells cut off a small part of each lamin A molecule, which allows it to carry out its function properly. In progeria, the molecules remain unaltered and interfere with the workings of the nuclear membrane until it starts to bulge and deform and the cell stops looking like a ball and resembles a sick popcorn kernel.
For the first time, the team recorded the extent to which progeria affects the mitochondria, which are the powerplants of the cell. The progeria defect causes them to swell, fragment, and malfunction. This was suspected, but not properly seen before.
But the bigger discovery by the UMD team is that small doses of methylene blue, a common, inexpensive, water-soluble can reverse the symptoms of progeria in less than eight weeks.
First produced in 1876, methylene blue is used as a bacteriological stain, for treating plaque psoriasis and other diseases, in cases of cyanide poisoning, and even for cleaning aquaria because its toxicity is so low it won't harm fish eggs.
Working with human fibroblast cells, the team discovered that methylene blue changed progeria-affected cells from deformed to normal round shapes in a matter of weeks and almost completely repaired the damage until they are nearly indistinguishable from normal cells. Exactly how methylene blue does this is still not clear, but the team reports that repeated experiments have not revealed a single failure.
According to the team, methylene blue can also reverse the effects of normal aging in healthy cells, including damage to the mitochondria. The researchers say that the next step will be to test the chemical on animal models. If the results pan out, it could be used as an inexpensive disease treatment, as well as in non-prescription anti-aging treatments, cosmetics, and nutritional supplements.
"So far, we have done all of our work in stem cell lines." says Kan Cao, senior author on the study and an associate professor of cell biology and molecular genetics at UMD. "It is critical to see whether the effect extends to whole animals. We also want to see if methylene blue can repair specific effects of progeria in various cell types, such as bone, skin, cardiovascular cells and others. Further down the line, other groups might begin human clinical trials. It's very exciting."
The study was published in Aging Cell.Source: