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Cellular and Molecular Biology of Neuronal Dystonin.
MedLine Citation:
PMID:  23273860     Owner:  NLM     Status:  Publisher    
Neuronal dystonin isoforms are giant cytoskeletal cross-linking proteins capable of interacting with actin and microtubule networks, protein complexes, membrane-bound organelles and cellular membranes. In the neuromuscular system, dystonin proteins are involved in maintaining cytoarchitecture integrity and have more recently been ascribed roles in other cellular processes such as organelle structure and intracellular transport. Loss of dystonin expression in mice results in a profound sensory ataxia termed dystonia musculorum (dt), which is attributed to the degeneration of sensory nerves. This chapter provides a comprehensive overview of the dystonin gene, the structure of encoded proteins, biological functions of neuronal dystonin isoforms, and known roles of dystonin in dt pathogenesis and human disease.
Andrew Ferrier; Justin G Boyer; Rashmi Kothary
Publication Detail:
Journal Detail:
Title:  International review of cell and molecular biology     Volume:  300C     ISSN:  1937-6448     ISO Abbreviation:  Int Rev Cell Mol Biol     Publication Date:  2013  
Date Detail:
Created Date:  2012-12-31     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101475846     Medline TA:  Int Rev Cell Mol Biol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  85-120     Citation Subset:  -    
Copyright Information:
Copyright © 2013 Elsevier Inc. All rights reserved.
Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, Ontario, Canada; Department of Cellular and Molecular Medicine, University of Ottawa, Ottawa, Ontario, Canada.
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