Microtubule binding distinguishes dystrophin from utrophin
نویسندگان
چکیده
منابع مشابه
Utrophin lacks the rod domain actin binding activity of dystrophin.
We previously identified a cluster of basic spectrin-like repeats in the dystrophin rod domain that binds F-actin through electrostatic interactions (Amann, K. J., Renley, B. A., and Ervasti, J. M. (1998) J. Biol. Chem. 273, 28419-28423). Because of the importance of actin binding to the presumed physiological role of dystrophin, we sought to determine whether the autosomal homologue of dystrop...
متن کاملStructure-function relationships in dystrophin and utrophin.
Introduction The integrity of the animal cell membrane is believed to be maintained in part by the large (approx. 400 kDa) cytoskeletal proteins dystrophin and utrophin. Both proteins are capable of forming a link, probably flexible and potentially extensible/compressible, between the actin cytoskeleton and the cell membrane itself. Utrophin is expressed in all cell types, whereas dystrophin ex...
متن کاملIdentification of α - Syntrophin Binding to Syntrophin Triplet , Dystrophin , and Utrophin
Syntrophin represents three cytoplasmic components of the dystrophin-glycoprotein complex that links the cytoskeleton to the extracellular matrix in skeletal muscle. α-Syntrophin has now been translated in vitro and shown to associate directly with all three components of the syntrophin triplet and with dystrophin. The in vitro translated 71-kDa non-muscle dystrophin isoform, containing the cys...
متن کاملThe dystrophin / utrophin homologues in Drosophila and in sea urchin.
The gene which is defective in Duchenne muscular dystrophy (DMD) is the largest known gene containing at least 79 introns, some of which are extremely large. The product of the gene in muscle, dystrophin, is a 427 kDa protein. The same gene encodes at least two additional non-muscle full length dystrophin isoforms transcribed from different promoters located in the 5'-end region of the gene, an...
متن کاملFunctional Substitution by TAT-Utrophin in Dystrophin-Deficient Mice
BACKGROUND The loss of dystrophin compromises muscle cell membrane stability and causes Duchenne muscular dystrophy and/or various forms of cardiomyopathy. Increased expression of the dystrophin homolog utrophin by gene delivery or pharmacologic up-regulation has been demonstrated to restore membrane integrity and improve the phenotype in the dystrophin-deficient mdx mouse. However, the lack of...
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ژورنال
عنوان ژورنال: Proceedings of the National Academy of Sciences
سال: 2014
ISSN: 0027-8424,1091-6490
DOI: 10.1073/pnas.1323842111