Progerin作用的伴侣蛋白和核纤层蛋白间的相互作用:在早老症中Me118与emerin的相互作用(英文)  

Association of progerin-interactive partner proteins with lamina proteins:Mel18 is associated with emerin in HGPS

作　　者：W. Ted BROWN Nanbert ZHONG 

机构地区：[1]Department of Human Genetics, New York State Institute for Basic Research in Developmental Disabilities

出　　处：《北京大学学报（医学版）》2009年第4期397-401,共5页Journal of Peking University:Health Sciences

摘　　要：Objective:The Hutchinson-Gilford progeria syndrome (HGPS or progeria) is a childhood disorder with features of premature aging and is caused by mutations in the lamin A gene resulting in the production of an abnormal protein,termed progerin. To investigate the underlying pathogenic mechanism,we studied the nuclear co-localization and association of progerin interactive partner proteins (PIPPs) with lamina proteins. Methods:Both wild-type (WT) and progeria fibroblasts were studied by various methods including confocal microscopy,immunoprecipitation and Western blot.Results:All PIPPs discovered so-far co-localized with lamin A/C. In addition,the PIPPs were selectively associated with lamina proteins. An increased immunofluorescent staining signal was found for Mel18 in HGPS as compared to WT cells. An association of Mel18 with emerin was observed in HGPS,but not in WT cells. Conclusion:Based on these findings,we propose that PIPPs,along with associated lamina proteins may form a pathogenic progerin-containing protein complex.Objective：The Hutchinson-Gilford progeria syndrome （HGPS or progeria） is a childhood disorder with features of premature aging and is caused by mutations in the lamin A gene resulting in the production of an abnormal protein,termed progerin. To investigate the underlying pathogenic mechanism,we studied the nuclear co-localization and association of progerin interactive partner proteins （PIPPs） with lamina proteins. Methods：Both wild-type （WT） and progeria fibroblasts were studied by various methods including confocal microscopy,immunoprecipitation and Western blot.Results：All PIPPs discovered so-far co-localized with lamin A/C. In addition,the PIPPs were selectively associated with lamina proteins. An increased immunofluorescent staining signal was found for Mel18 in HGPS as compared to WT cells. An association of Mel18 with emerin was observed in HGPS,but not in WT cells. Conclusion：Based on these findings,we propose that PIPPs,along with associated lamina proteins may form a pathogenic progerin-containing protein complex.

关 键 词：早衰 核纤层蛋白A型 核被膜 核纤层 

分 类 号：R725.8[医药卫生—儿科]