Expression of dystrophin associated glycoprotein complex in Duchenne muscular dystrophy at different age groups
10.3969/j.issn.1000-3606.2009.11.010
- VernacularTitle:不同年龄阶段Duchenne型肌营养不良患儿肌细胞膜上DGC关联复合物蛋白的表达
- Author:
Xihua LI
;
Ling XU
;
Xiaoqing LIU
;
Lei ZHAO
;
Yan WU
;
Ying SHENG
;
Jie WU
;
Xuefang GU
- Publication Type:Journal Article
- Keywords:
Duchnne muscular dystrophy;
dystrophin;
dystrophin related DGC proteins
- From:
Journal of Clinical Pediatrics
2009;(11):1040-1044
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze the expression of dystrophin associated glycoprotein complex (DGC) in Duchnne muscular dystrophy (DMD) in different age groups. Methods The confirmed 24 DMD patients were divided into 3 groups according to their chronological age (children whose age between 1 to 3 were assigned to early childhood group; children whose age between 3 to 6 were assigned to preschool group; children whose age between 6 to 13 were assigned to school-age group). Several proteins from sarcolemmal muscle membrane were analyzed by immunohistocbemistry. Results The primary loss of dystrophin may lead to a secondary or completely deficiency of α-SG, β-SG, γ-SG,δ-SG, β-DG and dysferlin from sarcolemmal muscle membrane in different age groups. The expression of nNOS is completely deficient in the 3 groups, while the expression of caveolin-3 increased. Conclusions The expression of dystrophin related DGC proteins from DMD patients in different age groups showed diversed degrees of deficiency or complete deficiency. The deficiency of these proteins may occur during the embryonic period, while the increased expression of eaveolin-3 may have a relation with the regeneration of skeletal muscle fibers.
