A ROLE FOR MAST-CELLS IN THE PROGRESSION OF DUCHENNE MUSCULAR-DYSTROPHY - CORRELATIONS IN DYSTROPHIN-DEFICIENT HUMANS, DOGS, AND MICE

被引:82
作者
GOROSPE, JRM
THARP, MD
HINCKLEY, J
KORNEGAY, JN
HOFFMAN, EP
机构
[1] UNIV PITTSBURGH,SCH MED,DEPT MOLEC GENET & BIOCHEM,PITTSBURGH,PA 15261
[2] UNIV PITTSBURGH,SCH MED,DEPT HUMAN GENET,PITTSBURGH,PA 15261
[3] UNIV PITTSBURGH,SCH MED,DEPT PEDIAT,PITTSBURGH,PA 15261
[4] UNIV PITTSBURGH,SCH MED,DEPT DERMATOL,PITTSBURGH,PA 15261
[5] N CAROLINA STATE UNIV,COLL VET MED,DEPT COMPAN ANIM & SPECIAL SPECIES MED,RALEIGH,NC
关键词
MAST CELLS; DUCHENNE MUSCULAR DYSTROPHY; DYSTROPHIN;
D O I
10.1016/0022-510X(94)90050-7
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Dystrophin deficiency has been shown to be the underlying cause of Duchenne muscular dystrophy. Although dystrophin-deficient homologous animal models have been identified (dog, mouse, and cat), the clinical expression of the biochemical defect is species-specific. Thus, while the genetics and biochemistry of Duchenne dystrophy is understood, the pathophysiological cascade leading to muscle weakness in only humans and dogs remains obscure. To begin to dissect the pathophysiology at the histological level, we undertook a systematic study of mast cells in normal and dystrophin-deficient muscle. Mast cells have been implicated in the development of fibrosis in other disorders, and progressive fibrosis has been hypothesized to mediate the failure of muscle regeneration in human and dog dystrophin deficiency. Our results show a strong correlation between mast cell content and localization, and the clinico-histopathological progression in humans, dogs and mice. The mast cell increases were disease specific: other dystrophic myopathies with normal dystrophin generally did not show substantial increases in mast cell content or degranulation. Our data suggest that mast cell accumulation and degranulation may cause the grouped necrosis characteristic of dystrophin deficiency in all species.
引用
收藏
页码:44 / 56
页数:13
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