Article
[A-type lamins and progeroïd syndromes : persistent farnesylation with dramatic effects].
Medecine sciences : M/S - 1 Oct 2008
Navarro Claire L, Poitelon Yannick, 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 heterogeneous disorders are caused by altered Lamin maturation pathway. In physiological conditions, mature Lamin A is obtained...
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