نتایج جستجو برای: gilford progeria syndrome

تعداد نتایج: 622251  

Journal: :Frontiers in bioscience 2011
Laia Trigueros-Motos Jose M Gonzalez Jose Rivera Vicente Andres

Hutchinson-Gilford Progeria Syndrome (HGPS), a rare human disease characterized by premature aging, is mainly caused by the abnormal accumulation of progerin, a mutant form of the mammalian nuclear envelope component lamin A. HGPS patients exhibit vascular alterations and die at an average age of 13 years, predominantly from myocardial infarction or stroke. Animal models of HGPS have been a val...

Journal: :Journal of cell science 1991
A Colige B Nusgens C M Lapiere

The Hutchinson-Gilford syndrome (progeria) is a rare disorder in childhood characterized by premature and accelerated aging. This study reports the effect of a potent growth factor, EGF, on the proliferative capacities and extracellular matrix macromolecules and collagenase expression of two strains of progeria skin-derived cells. At low population doubling levels (PDL less than 10), confluent ...

Journal: :Circulation 2016
Leslie B Gordon Monica E Kleinman Joe Massaro Ralph B D'Agostino Heather Shappell Marie Gerhard-Herman Leslie B Smoot Catherine M Gordon Robert H Cleveland Ara Nazarian Brian D Snyder Nicole J Ullrich V Michelle Silvera Marilyn G Liang Nicolle Quinn David T Miller Susanna Y Huh Anne A Dowton Kelly Littlefield Maya M Greer Mark W Kieran

BACKGROUND Hutchinson-Gilford progeria syndrome is an extremely rare, fatal, segmental premature aging syndrome caused by a mutation in LMNA yielding the farnesylated aberrant protein progerin. Without progerin-specific treatment, death occurs at an average age of 14.6 years from an accelerated atherosclerosis. A previous single-arm clinical trial demonstrated that the protein farnesyltransfera...

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