Expression of Plasminogen Activator Inhibitor-1 of Frozen Muscle Specimensin Muscular Dystrophy
- VernacularTitle:肌营养不良冷冻肌肉标本纤溶酶原激活物抑制物-1的表达
- Author:
gui-lian, SUN
;
hong-kun, JIANG
;
shuang, ZHAO
;
jing, ZHANG
- Publication Type:Journal Article
- Keywords:
plasminogen activator inhibitor-1;
muscular dystrophy;
urokinase plasminogen activator system
- From:Journal of Applied Clinical Pediatrics
2006;0(24):-
- CountryChina
- Language:Chinese
-
Abstract:
Objective To explore the role of plasminogen activator inhibitor-1(PAI-1)in development of progressive fibrosis via the inhibition of extracellular matrix degradation,and to reveal the contributive role of PAI-1 in muscular dystrophy(MD).Methods Expression and cellular localization of PAI-1 protein were examined in frozen muscle specimens obtained via biopsy from 5 patients with duchenne muscular dystrophy(DMD),3 patients with becker muscular dystrophy(BMD),9 patients with congenital muscular dystrophy(CMD) and 4 cases with normal muscle by immunohistochemistry,double immunofluorescence and Western-blot analysis.Results PAI-1 was positive only in vascular endothelial cells of normal muscle.Both immunohistochemistry and Western-blot analysis showed that PAI-1 expression distinctly increased in most dystrophic muscles of MD than that in normal muscles.Double immunolabeling revealed that PAI-1 strongly expressed in cytoplasm and nuclei of regenerating muscle fibers,macrophages,macrophage infiltrating necrotic fibers.Some activated fibroblasts in endomysium and perimysium of DMD and CMD muscles were positive for PAI-1.Conclusions The functional consequence of overexpression of PAI-1 in dystrophic muscles is unknown but the elevated local expression of PAI-1 in diseased muscles of MD and their distinct distribution pattern provide evidence that PAI-1 participate in pathogenesis of MD.
