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Genetic Engineering of Dystroglycan in Animal Models of Muscular Dystrophy
Joint Authors
Giardina, Bruno
Sciandra, Francesca
Bigotti, Maria Giulia
Bozzi, Manuela
Brancaccio, Andrea
Source
Issue
Vol. 2015, Issue 2015 (31 Dec. 2015), pp.1-10, 10 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2015-08-24
Country of Publication
Egypt
No. of Pages
10
Main Subjects
Abstract EN
In skeletal muscle, dystroglycan (DG) is the central component of the dystrophin-glycoprotein complex (DGC), a multimeric protein complex that ensures a strong mechanical link between the extracellular matrix and the cytoskeleton.
Several muscular dystrophies arise from mutations hitting most of the components of the DGC.
Mutations within the DG gene (DAG1) have been recently associated with two forms of muscular dystrophy, one displaying a milder and one a more severe phenotype.
This review focuses specifically on the animal (murine and others) model systems that have been developed with the aim of directly engineering DAG1 in order to study the DG function in skeletal muscle as well as in other tissues.
In the last years, conditional animal models overcoming the embryonic lethality of the DG knock-out in mouse have been generated and helped clarifying the crucial role of DG in skeletal muscle, while an increasing number of studies on knock-in mice are aimed at understanding the contribution of single amino acids to the stability of DG and to the possible development of muscular dystrophy.
American Psychological Association (APA)
Sciandra, Francesca& Bigotti, Maria Giulia& Giardina, Bruno& Bozzi, Manuela& Brancaccio, Andrea. 2015. Genetic Engineering of Dystroglycan in Animal Models of Muscular Dystrophy. BioMed Research International،Vol. 2015, no. 2015, pp.1-10.
https://search.emarefa.net/detail/BIM-1056225
Modern Language Association (MLA)
Sciandra, Francesca…[et al.]. Genetic Engineering of Dystroglycan in Animal Models of Muscular Dystrophy. BioMed Research International No. 2015 (2015), pp.1-10.
https://search.emarefa.net/detail/BIM-1056225
American Medical Association (AMA)
Sciandra, Francesca& Bigotti, Maria Giulia& Giardina, Bruno& Bozzi, Manuela& Brancaccio, Andrea. Genetic Engineering of Dystroglycan in Animal Models of Muscular Dystrophy. BioMed Research International. 2015. Vol. 2015, no. 2015, pp.1-10.
https://search.emarefa.net/detail/BIM-1056225
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1056225