Investigation of age-related changes in LMNA splicing and expression of progerin in human skeletal muscles.
Luo, Yue-Bei; Mitrpant, Chalermchai; Johnsen, Russell D; Fabian, Victoria A; Fletcher, Sue; Mastaglia, Frank L; Wilton, Steve D.
Int J Clin Exp Pathol
; 6(12): 2778-86, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-24294364
Progeria: a paradigm for translational medicine.
Dangerous Entrapment for NRF2.
Lamin A, farnesylation and aging.
Proliferation of progeria cells is enhanced by lamina-associated polypeptide 2α (LAP2α) through expression of extracellular matrix proteins.
Emerging candidate treatment strategies for Hutchinson-Gilford progeria syndrome.
Vascular Smooth Muscle-Specific Progerin Expression Accelerates Atherosclerosis and Death in a Mouse Model of Hutchinson-Gilford Progeria Syndrome.
Expression of progerin does not result in an increased mutation rate.
New Lmna knock-in mice provide a molecular mechanism for the 'segmental aging' in Hutchinson-Gilford progeria syndrome.
Interplay of primary sequence, position and secondary RNA structure determines alternative splicing of LMNA in a pre-mature aging syndrome.
Accumulation of the inner nuclear envelope protein Sun1 is pathogenic in progeric and dystrophic laminopathies.