Article
Altered splicing in prelamin A-associated premature aging phenotypes.
Progress in molecular and subcellular biology - 1 Jan 2006
De Sandre-Giovannoli Annachiara, Lévy Nicolas
Abstract excerpt
Hutchinson-Gilford progeria (HGPS), a rare and severe developmental disorder characterized by features recalling premature aging, and restrictive dermopathy (RD), a neonatal lethal genodermatosis, have recently been identified as being primary or secondary "laminopathies." These are heterogeneous disorders due to altered function of lamins A/C or related proteins. In physiological conditions, mature lamin A is...
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