Current Trends in Engineering Science
[ ISSN : 2833-356X ]
Hutchinson-Gilford Progeria Syndrome: Premature Aging
Marie Curie Science Research Center, France
Corresponding Authors
Keywords
Abstract
Hutchinson–Gilford Progeria Syndrome (HGPS) is a rare fetal disorder that causes children to age faster than normal. This disorder results from a point mutation in the LMNA gene resulting in production of progeria, an abnormal form of Lamin A. Progeria damages the nuclear structure. As a result, cells in HGPS patients experience DNA damage, altered chromatin structure, and epigenetic dysregulation. Children with HGPS typically experience hair loss, thin skin and early heart disease. While there is still no cure for this disease, newer techniques such as RNA based treatments show encouraging progress. Studying HGPS continues to deepen our understanding of the molecular pathway involved both pathological and normal aging.