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Rare Diseases Week Premature Ageing
25 February 2014

Premature Ageing

Hutchinson-Gilford progeria syndrome, also known as progeria, is an extremely rare genetic disorder that causes children to suffer symptoms of accelerated ageing. It’s caused by a mutation in the gene that encodes a protein called lamin A, which provides structural support for the cell’s control centre or nucleus. With the faulty protein, nuclei are misshapen and unstable (right), unlike the spherical shape of a healthy nucleus (left). The disorder is invariably fatal, with most patients dying of heart disease in their teens. But clinical trials have recently returned encouraging, if mixed, results for a repurposed cancer drug called lonafarnib, which is thought to deactivate the defective scaffolding protein. Nine of the 25 trial subjects gained weight – something progeria patients struggle with – while 18 experienced improvements in the flexibility of blood vessels, potentially reducing the risk of heart attack and stroke. A second trial is ongoing.

Written by Daniel Cossins

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BPoD stands for Biomedical Picture of the Day. Managed by the MRC Laboratory of Medical Sciences until Jul 2023, it is now run independently by a dedicated team of scientists and writers. The website aims to engage everyone, young and old, in the wonders of biology, and its influence on medicine. The ever-growing archive of more than 4000 research images documents over a decade of progress. Explore the collection and see what you discover. Images are kindly provided for inclusion on this website through the generosity of scientists across the globe.

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