Mzwanele Ngubo, Zhaoyi Chen, Darin McDonald, Rana Karimpour, Amit Shrestha, Julien Yockell-Lelièvre, Aurélie Laurent, Ojong Tabi Ojong Besong, Eve C Tsai, F Jeffrey Dilworth, Michael J Hendzel, William L Stanford
Hutchinson-Gilford Progeria syndrome (HGPS) is a lethal premature aging disorder caused by a de novo heterozygous mutation that leads to the accumulation of a splicing isoform of Lamin A termed progerin. Progerin expression deregulates the organization of the nuclear lamina and the epigenetic landscape. Progerin has also been observed to accumulate at low levels during normal aging in cardiovascular cells of adults that do not carry genetic mutations linked with HGPS. Therefore, the molecular mechanisms that lead to vascular dysfunction in HGPS may also play a role in vascular aging-associated diseases, such as myocardial infarction and stroke...
April 4, 2024: Aging Cell