Lamin A, progeria and aging research

Source:  Lamin A, progeria and aging research    Tag:  what causes progeria disease
New clues for treatment of disease that causes accelerated aging Washington U. in St. Louis 5/2/07 "...Scientists studying the genes of two infants who died of mysterious illnesses found the infants had mutations in LMNA, the same gene altered in patients with the premature aging condition progeria. But the infants' unusual mutations caused them to make many more bad copies of the gene's primary protein, lamin A, than progeria patients..."Our success in treating these cells, which had unusually high levels of bad lamin A, suggest that progeria treatment may not be as distant as we thought," says senior author Jeffrey Miner, Ph.D., ... "If physicians can reduce production of bad lamin A by as little as half in progeria patients, we might see significant improvement." ...Progeria treatment also has potential implications for larger populations. The LMNA gene is involved in several other more prevalent disorders including forms of muscular dystrophy and heart disease...In addition, recent studies by other labs have shown that occasional errors in the production of lamin A may take place even in people with "normal" copies of the gene. Scientists suspect that accumulation of these bad copies may contribute to aging. If so, treatments that work for progeria patients may one day be adapted to reduce the effects of aging...Science still has much to learn about lamin A. When appropriately produced, the protein becomes part of a meshwork between DNA and the nuclear membrane, which keeps DNA in a pocket known as the nucleus. Lamin A may help arrange the DNA in a way that affects how often genes are used to make proteins...To get to where it does its job, lamin A binds to another molecule that guides it to the nuclear membrane. Normally the portion of lamin A bound to the guide molecule gets cut off at this point, freeing the rest of lamin A to become part of the nearby meshwork where it belongs. In most patients with progeria, though, the spot in lamin A where the guide molecule gets clipped off is erroneously deleted during initial assembly of the protein. That leaves lamin A stuck to its guide molecule, which in turn is stuck in the nuclear membrane. Scientists believe this misplacement causes progeria; under the microscope, it leads to visible distortions in the structure of cell nuclei..."It's a matter of ratios: the more abnormal protein you have, the more severe the disease," Miner says. "And apparently we don't have that far to go to tip that ratio in progeria patients' favor." "

There has been debate over whether research to help victims of progeria might possibly help aging and longevity for healthy folks. It appears from this that it might. So add lamin A to your Google Alerts.

More on lamins at NIH and Ouroboros (excellent blog on biology of aging)

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