Dystrophins, Utrophins, and Associated Scaffolding Complexes: Role in Mammalian Brain and Implications for Therapeutic Strategies
Joint Authors
Vaillend, Cyrille
Perronnet, Caroline
Source
Issue
Vol. 2010, Issue 2010 (31 Dec. 2009), pp.1-19, 19 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2010-04-26
Country of Publication
Egypt
No. of Pages
19
Main Subjects
Abstract EN
Two decades of molecular, cellular, and functional studies considerably increased our understanding of dystrophins function and unveiled the complex etiology of the cognitive deficits in Duchenne muscular dystrophy (DMD), which involves altered expression of several dystrophin-gene products in brain.
Dystrophins are normally part of critical cytoskeleton-associated membrane-bound molecular scaffolds involved in the clustering of receptors, ion channels, and signaling proteins that contribute to synapse physiology and blood-brain barrier function.
The utrophin gene also drives brain expression of several paralogs proteins, which cellular expression and biological roles remain to be elucidated.
Here we review the structural and functional properties of dystrophins and utrophins in brain, the consequences of dystrophins loss-of-function as revealed by numerous studies in mouse models of DMD, and we discuss future challenges and putative therapeutic strategies that may compensate for the cognitive impairment in DMD based on experimental manipulation of dystrophins and/or utrophins brain expression.
American Psychological Association (APA)
Perronnet, Caroline& Vaillend, Cyrille. 2010. Dystrophins, Utrophins, and Associated Scaffolding Complexes: Role in Mammalian Brain and Implications for Therapeutic Strategies. BioMed Research International،Vol. 2010, no. 2010, pp.1-19.
https://search.emarefa.net/detail/BIM-989149
Modern Language Association (MLA)
Perronnet, Caroline& Vaillend, Cyrille. Dystrophins, Utrophins, and Associated Scaffolding Complexes: Role in Mammalian Brain and Implications for Therapeutic Strategies. BioMed Research International No. 2010 (Dec. 2010), pp.1-19.
https://search.emarefa.net/detail/BIM-989149
American Medical Association (AMA)
Perronnet, Caroline& Vaillend, Cyrille. Dystrophins, Utrophins, and Associated Scaffolding Complexes: Role in Mammalian Brain and Implications for Therapeutic Strategies. BioMed Research International. 2010. Vol. 2010, no. 2010, pp.1-19.
https://search.emarefa.net/detail/BIM-989149
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-989149