Brighton researchers create first genetically stable, lab-grown stem cell models of Progeria, paving the way for research in heart ageing and rare diseases
Hutchinson-Gilford Progeria Syndrome (HGPS) is a fatal, extremely rare condition affecting around one in 20 million children worldwide. It is caused by a gene mutation which produces a faulty protein called progerin – which accelerates cell ageing, leading to severe cardiovascular disease and heart failure in affected children, who often die in their early teens.
Until now access to human progeria cell lines often were produced with non-stable methods that would cause the potential of DNA mutations which would therefore mean downstream analysis would not be correct.
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