When 8-year olds look like 80: Researchers describe mechanism behind premature aging disease

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.

Progerin: the protein behind the aging disease progeria

Progerin, a protein present in very high concentration in progeria cells, is known to be responsible for many of the characteristics of the disease. It is a mutant version of lamin A, a protein crucial for the stability of the nucleus and involved in many essential nuclear functions. How progerin exerts its effects exactly is the reseach interest of Roland Foisner and his team at the Max F. Perutz Laboratories -- a joint venture of the University of Vienna and the Medical University of Vienna. They investigate the molecular functions of nuclear lamins and their mutated forms such as progerin and associated diseases.

"A few years ago, we and others found that progeria cells have much less LAP2α than normal cells. LAP2α is a protein that interacts with lamin A to regulate cell proliferation, the process that produces new cells. Interestingly, LAP2α levels also decrease during normal aging," explains Roland Foisner, Deputy Director of the Department of Medical Biochemistry of the Medical University of Vienna. Supported by an Innovator Award from The Progeria Research Foundation, senior postdoc Thomas Dechat and PhD student Sandra Vidak in collaboration with Tom Misteli from the NIH National Cancer Institute (USA) developed a cell line that allows studying the molecular mechanisms behind progeria in the lab.

See more: https://www.sciencedaily.com/releases/2015/10/151007033313.htm

Source: Medical University of Vienna

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