title: Association of progerin-interactive partner proteins with lamina proteins: Mel18 is associated with emerin in HGPS.
abstract: 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.
  
  
  CONCLUSIONS
  Based on these findings, we propose that PIPPs, along with associated lamina proteins may form a pathogenic progerin-containing protein complex.
authors.affiliation: Department of Human Genetics, New York State Institute for Basic Research in Developmental Disabilities, Staten Island, NY 10314, USA.
authors.forename: Wei-na
authors.initials: WN
authors.lastname: Ju
authors.forename: W Ted
authors.initials: WT
authors.lastname: Brown
authors.forename: Nanbert
authors.initials: N
authors.lastname: Zhong
mesh_terms: D006801:Humans; D034904:Lamin Type A / Q000378:metabolism*; D008565:Membrane Proteins / Q000378:metabolism*; D009154:Mutation; D034881:Nuclear Lamina / Q000378:metabolism; D009687:Nuclear Proteins / Q000235:genetics / Q000378:metabolism*; D063150:Polycomb Repressive Complex 1; D011371:Progeria / Q000378:metabolism* / Q000473:pathology; D011485:Protein Binding; D011498:Protein Precursors / Q000235:genetics / Q000378:metabolism*; D012097:Repressor Proteins / Q000378:metabolism*
chemical_list: D034904:Lamin Type A; D008565:Membrane Proteins; D009687:Nuclear Proteins; C472312:PCGF6 protein, human; D011498:Protein Precursors; D012097:Repressor Proteins; C092710:emerin; C090764:prelamin A; D063150:Polycomb Repressive Complex 1