Effect of progerin on the accumulation of oxidized proteins in fibroblasts from Hutchinson Gilford progeria patients

摘要: The mutation responsible for Hutchinson Gilford Progeria Syndrome (HGPS) causes abnormal nuclear morphology. Previous studies show that free radicals and reactive oxygen species play major roles in the etiology and/or progression of neurodegenerative diseases aging. This study compares oxidative stress responses between progeric normal fibroblasts. Our data revealed higher ROS levels HGPS cells compared to age-matched controls. In response challenge, showed increased mRNA mitochondrial superoxide dismutase (SOD) SOD protein content. However, this did not prevent a drop ATP content progeria have shown declines human fibroblast interfere with programmed cell death promote necrotic inflammation. Notably, our investigations fibroblasts was only approximately 50% found healthy Furthermore, analysis decrease total caspase-like proteasome activity two active proteolytic complex subunits (beta(5) beta(7)). A number indicate molecular mechanisms causing accelerated aging patients also occur older individuals. Thus, results may help explain some cellular changes accompany