Association of progerin-interactive partner proteins with lamina proteins:Mel18 is associated with emerin in HGPS / 北京大学学报(医学版)
Journal of Peking University(Health Sciences)
;
(6): 397-401, 2009.
Artículo
en Chino
| WPRIM
| ID: wpr-405903
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 mecha-nism, 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 eonfocal microscopy, immunopreeipitation 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 com-pared to WT cells. An association of Me118 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.
Texto completo:
Disponible
Índice:
WPRIM (Pacífico Occidental)
Idioma:
Chino
Revista:
Journal of Peking University(Health Sciences)
Año:
2009
Tipo del documento:
Artículo
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