共 356 条
Cell-matrix interactions in muscle disease
被引:67
作者:

Carmignac, Virginie
论文数: 0 引用数: 0
h-index: 0
机构:
Lund Univ, Dept Expt Med Sci, Muscle Biol Unit, S-22184 Lund, Sweden Lund Univ, Dept Expt Med Sci, Muscle Biol Unit, S-22184 Lund, Sweden

Durbeej, Madeleine
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h-index: 0
机构:
Lund Univ, Dept Expt Med Sci, Muscle Biol Unit, S-22184 Lund, Sweden Lund Univ, Dept Expt Med Sci, Muscle Biol Unit, S-22184 Lund, Sweden
机构:
[1] Lund Univ, Dept Expt Med Sci, Muscle Biol Unit, S-22184 Lund, Sweden
关键词:
muscle;
extracellular matrix;
membrane;
cytoskeleton;
disease;
muscular dystrophy;
CONGENITAL MUSCULAR-DYSTROPHY;
COLLAGEN-VI DEFICIENCY;
LAMININ ALPHA-1 CHAIN;
BONE-MARROW-TRANSPLANTATION;
MYOSTATIN PROPEPTIDE GENE;
IMPROVES CARDIAC-FUNCTION;
TRANSGENIC MDX MICE;
DY(W) MOUSE MODEL;
SKELETAL-MUSCLE;
STEM-CELLS;
D O I:
10.1002/path.3020
中图分类号:
R73 [肿瘤学];
学科分类号:
100214 ;
摘要:
The extracellular matrix (ECM) provides a solid scaffold and signals to cells through ECM receptors. The cell-matrix interactions are crucial for normal biological processes and when disrupted they may lead to pathological processes. In particular, the biological importance of ECM-cell membrane-cytoskeleton interactions in skeletal muscle is accentuated by the number of inherited muscle diseases caused by mutations in proteins conferring these interactions. In this review we introduce laminins, collagens, dystroglycan, integrins, dystrophin and sarcoglycans. Mutations in corresponding genes cause various forms of muscular dystrophy. The muscle disorders are presented as well as advances toward the development of treatment. Copyright (C) 2011 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
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页码:200 / 218
页数:19
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