... attractive approach is to increase utrophin expression. Utrophin shares considerable sequence, structural and functional similarity with dystrophin. Here, we test the hypothesis that utrophin also brings nNOS to the sarcolemma. Full-length utrophin cDNA was expressed in dystrophin-deficient mdx mice...

... Nitric oxide synthase Aquaporin 4 Syntrophin Muscular dystrophy Utrophin In skeletal muscle, the absence of a functional form of the protein dystrophin results in severe muscular dystrophy ( Hoffman et al., 1987 ). Dystrophin localizes to the sarcolemma and stabilizes a complex of proteins...

... by laminin polymerization. Assembly of one, a densely-distributed reticular matrix, was accompanied by a redistribution of cell-surface dystroglycan and cytoskeletal utrophin into matrix-receptor-cytoskeletal complexes. The other, a fibrillar matrix,accumulated in separate zones associated with pre-existing...

.... Müller glial cells from rat retina express dystrophin protein Dp71, utrophin and the members of the dystrophin-associated glycoprotein complex (DGC), namely β-dystroglycan, δ- and γ-sarcoglycans and α1-syntrophin. The DGC could function in muscle as a link between the cystoskeleton and the extracellular...

... for correspondence (e-mail: [email protected] ) 10 07 2000 22 05 2000 © 2000 by Company of Biologists 2000 Dystrobrevin Dystrophin Utrophin Syntrophin Dp71 Kidney Epithelium Dystrophin, the product of the Duchenne muscular dystrophy (DMD) gene, is part...

... receptors and another dystrophin-related protein, utrophin. This redistribution is not associated with tyrosine phosphorylation of α-dystrobrevin-1 by agrin. Furthermore, we show that α-dystrobrevin-1 is associated with both utrophin in C2 cells and dystrophin in mature skeletal muscle. Thus α-dystrobrevin...

... dystrophy. Mice showed mild pathology in accessory extraocular muscles, but no signs of pathology were evident in the principal extraocular muscles at any age. By immunoblotting, the extraocular muscles of mdx mice exhibited increased levels of a dystrophin analog, dystrophin-related protein or utrophin...

...Tejvir S. Khurana; Louis M. Kunkel; Alan D. Frederickson; Salvatore Carbonetto; Simon C. Watkins ABSTRACT Dystrophin-related protein/utrophin is a large, cytoskeletal protein that shares significant sequence similarity with dys-trophin. Dystrophin-related protein is known to be enriched where cell...

...S. J. Winder; L. Hemmings; S. K. Maciver; S. J. Bolton; J. M. Tinsley; K. E. Davies; D. R. Critchley; J. Kendrick-Jones ABSTRACT Utrophin, or dystrophin-related protein, is an autosomal homologue of dystrophin. The protein is apparently ubiq-uitously expressed and in muscle tissues the expression...

... analysis of immunoblots of adult mdx muscle samples showed an increase of approximately 200% for vinculin and talin, cytoskeletal proteins that mediate thin filament-membrane interactions at myotendinous junctions. Blots also showed an increase (143%) in the dys- trophin-related protein called utrophin...