Review
doi: 10.1097/00019052-199710000-00014.
Functions of dystrophin and dystrophin associated proteins
Affiliations
- PMID: 9330892
- DOI: 10.1097/00019052-199710000-00014
Item in Clipboard
Review
Functions of dystrophin and dystrophin associated proteins
Curr Opin Neurol.
1997 Oct.
Abstract
Dystrophin is a protein product of the X-linked gene mutation that is responsible for Duchenne and Becker muscular dystrophies. The protein binds actin and associates with dystrophin-glycoprotein complex to link the cytoskeleton to the extracellular matrix. Defects in the components of the dystrophin-glycoprotein complex are responsible for several phenotypes of muscular dystrophy.
Similar articles
-
The role of the dystrophin-glycoprotein complex in the molecular pathogenesis of muscular dystrophies.Neuromuscul Disord. 1993 Sep-Nov;3(5-6):533-5. doi: 10.1016/0960-8966(93)90110-6. Neuromuscul Disord. 1993. PMID: 8186706 Review.
-
Dystrophin-glycoprotein complex: its role in the molecular pathogenesis of muscular dystrophies.Muscle Nerve. 1994 Jan;17(1):2-15. doi: 10.1002/mus.880170103. Muscle Nerve. 1994. PMID: 8264699 Review.
-
Dystrophin and related proteins.Curr Opin Genet Dev. 1993 Jun;3(3):484-90. doi: 10.1016/0959-437x(93)90124-8. Curr Opin Genet Dev. 1993. PMID: 8353425 Review.
-
Differential effects of dystrophin and utrophin gene transfer in immunocompetent muscular dystrophy (mdx) mice.Physiol Genomics. 2000 Sep 8;3(3):133-44. doi: 10.1152/physiolgenomics.2000.3.3.133. Physiol Genomics. 2000. PMID: 11015608
-
Gene therapy research for Duchenne and Becker muscular dystrophies.Curr Opin Neurol. 1997 Oct;10(5):430-5. doi: 10.1097/00019052-199710000-00013. Curr Opin Neurol. 1997. PMID: 9330891 Review.
Cited by
-
Novel inhibitor candidates of TRPV2 prevent damage of dystrophic myocytes and ameliorate against dilated cardiomyopathy in a hamster model.Oncotarget. 2018 Feb 8;9(18):14042-14057. doi: 10.18632/oncotarget.24449. eCollection 2018 Mar 6. Oncotarget. 2018. PMID: 29581825 Free PMC article.
-
The expression of dystrophin and alpha1-syntrophin during skeletal muscle regeneration.J Muscle Res Cell Motil. 2001;22(2):185-91. doi: 10.1023/a:1010553104341. J Muscle Res Cell Motil. 2001. PMID: 11519741
-
A mutation in dVps28 reveals a link between a subunit of the endosomal sorting complex required for transport-I complex and the actin cytoskeleton in Drosophila.Mol Biol Cell. 2005 May;16(5):2301-12. doi: 10.1091/mbc.e04-11-1013. Epub 2005 Feb 23. Mol Biol Cell. 2005. PMID: 15728719 Free PMC article.
-
Blockade of TRPV2 is a Novel Therapy for Cardiomyopathy in Muscular Dystrophy.Int J Mol Sci. 2019 Aug 7;20(16):3844. doi: 10.3390/ijms20163844. Int J Mol Sci. 2019. PMID: 31394715 Free PMC article. Review.
-
Guanine analogues enhance antisense oligonucleotide-induced exon skipping in dystrophin gene in vitro and in vivo.Mol Ther. 2010 Apr;18(4):812-8. doi: 10.1038/mt.2009.320. Epub 2010 Jan 19. Mol Ther. 2010. PMID: 20087314 Free PMC article.
Publication types
MeSH terms
Substances
LinkOut - more resources
Full Text Sources
Other Literature Sources
Medical