Article
Single-cell analysis of the progeria arterial wall reveals progerin-induced progressive, cell type-specific dysfunction and somatic mutation accumulation.
Genome medicine - 31 Jul 2026
Merino Lara G, Revêchon Gwladys, Subhash Santhilal, Stefani Fabiana, Whisenant Daniel, Skipitari Marianna, Giraud Quentin, Muhl Lars, Mocci Giuseppe, Björkegren Johan, Machtel Piotr, He Liqun, Betsholtz Christer, Eriksson Maria
Abstract excerpt
BACKGROUND: The premature aging disorder Hutchinson-Gilford Progeria Syndrome (HGPS) is caused by de novo LMNA mutations producing the aberrant Lamin A isoform progerin. HGPS patients die from cardiovascular disease, with their arteries showing extensive cellular and structural remodeling, but the mechanisms driving vascular dysfunction are not fully understood. METHODS: To define molecular processes underlying...
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