Researchers Describe Mechanism Behind Progeria

[Source:  Medical X-Press]
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Progeria, a premature aging disease, is the research focus of Roland Foisner’s team at the Max F. Perutz Laboratories of the University of Vienna and the Medical University of Vienna. Children suffering from progeria die at an average age of 14 to 15 years, often from heart attacks and strokes. So far, there is no cure for the disease, and though researchers identified the abnormal protein behind the disease – progerin – the exact way in which it causes the accelerated aging remains elusive. In their latest publication in Genes & Development, Roland Foisner and his group describe a yet unknown mechanism behind progeria that may provide new approaches for therapy.

Children suffering from progeria are born normal, but from age one to two their disease starts to resemble premature aging in some aspects. So by the time they reach their teens they have typical age-related conditions such as brittle bones, stiff joints and severe cardiovascular disease. In the end many die from strokes and heart attacks before reaching their twenties. Presently, there is no cure for progeria. Patients can be treated with drugs called FTIs (farnesyltransferase inhibitors), which were initially developed to treat cancer. These drugs improve some aspects of the disease, such as bone structure, arterial stiffness, and increase estimated lifespan by at least 1.6 years
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