... impact on elongation are unclear. We found that Dystroglycan (Dg) and Dystrophin (Dys) are involved in BM fibril deposition. Moreover, they also orient stress fibers, by acting locally and in parallel to Fat2. Importantly, Dg-Dys complex-mediated cell-autonomous control of F-actin fiber orientation...

.... ( 2006 ). Severe muscular dystrophy in mice that lack dystrophin and alpha7 integrin. J. Cell Sci. 119 , 2185 -2195. Schmalbruch, H. ( 1975 ). Segmental fiber breakdown and defects of the plasmalemma in diseased human muscles. Acta Neuropathol. 33 , 129 -141. Schwander, M., Leu, M., Stumm...

... and epithelial development. As an integral component of the Dystrophin-associated glycoprotein complex, Dg plays a central role in linking the ECM and the cytoskeleton. Disruption of this linkage in skeletal muscle leads to various types of muscular dystrophies. In epithelial cells, reduced expression of Dg...

... are fertile but show gonad migration defects in addition to the defects seen in homozygotes, indicating that DGN-1 function is dosage sensitive. Phenotypic analyses show that DGN-1 and dystrophin-associated protein complex (DAPC)components have distinct and independent functions, in contrast to the situation...

... in a dystrophic pathology through disconnecting the cytoskeleton from the extracellular matrix in skeletal muscle by reducing the level of dystrophin protein at the sarcolemma. This is thought to allow tearing of this membrane, which in turn leads to cell death. Surprisingly, we have found that the progressive...

...Michael J. Parsons; Isabel Campos; Elizabeth M. A. Hirst; Derek L. Stemple Muscular dystrophy is frequently caused by disruption of the dystrophin-glycoprotein complex (DGC), which links muscle cells to the extracellular matrix. Dystroglycan, a central component of the DGC, serves as a laminin...

...-spectrin to alpha actinin and dystrophin . J. Cell Biol . 109 , 1633 – 1641 . 10.1083/jcb.109.4.1633 Campbell , K. P. and Kahl , S. D. ( 1989 ). Association of dystrophin and an integral membrane glycoprotein . Nature 338 , 259 – 262 . 10.1038/338259a0 Campos-Ortega...

...Angela Clerk; Peter N. Strong; Caroline A. Sewry ABSTRACT Dystrophin, the 427 × 10 3 M r product of the Duchenne muscular dystrophy (DMD) gene, was studied in human foetal skeletal muscle from 9 to 26 weeks of gestation. Dystrophin could be detected from at least 9 weeks of gestation...