Article
Recapitulation of premature ageing with iPSCs from Hutchinson-Gilford progeria syndrome.
Nature - 14 Apr 2011
Liu Guang-Hui, Barkho Basam Z, Ruiz Sergio, Diep Dinh, Qu Jing, Yang Sheng-Lian, Panopoulos Athanasia D, Suzuki Keiichiro, Kurian Leo, Walsh Christopher, Thompson James, Boue Stephanie, Fung Ho Lim, Sancho-Martinez Ignacio, Zhang Kun, Yates John, Izpisua Belmonte Juan Carlos
Abstract excerpt
Hutchinson-Gilford progeria syndrome (HGPS) is a rare and fatal human premature ageing disease, characterized by premature arteriosclerosis and degeneration of vascular smooth muscle cells (SMCs). HGPS is caused by a single point mutation in the lamin A (LMNA) gene, resulting in the generation of progerin, a truncated splicing mutant of lamin A. Accumulation of progerin leads to various ageing-associated nuclear...
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