Nonmechanical Roles of Dystrophin and Associated Proteins in Exercise, Neuromuscular Junctions, and Brains
Dystrophin-glycoprotein complex (DGC) is an important structural unit in skeletal muscle that connects the cytoskeleton (f-actin) of a muscle fiber to the extracellular matrix (ECM). Several muscular dystrophies, such as Duchenne muscular dystrophy, Becker muscular dystrophy, congenital muscular dys...
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MDPI AG
2015-07-01
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| Series: | Brain Sciences |
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| Online Access: | http://www.mdpi.com/2076-3425/5/3/275 |
| _version_ | 1819207322060193792 |
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| author | Bailey Nichols Shin'ichi Takeda Toshifumi Yokota |
| author_facet | Bailey Nichols Shin'ichi Takeda Toshifumi Yokota |
| author_sort | Bailey Nichols |
| collection | DOAJ |
| description | Dystrophin-glycoprotein complex (DGC) is an important structural unit in skeletal muscle that connects the cytoskeleton (f-actin) of a muscle fiber to the extracellular matrix (ECM). Several muscular dystrophies, such as Duchenne muscular dystrophy, Becker muscular dystrophy, congenital muscular dystrophies (dystroglycanopathies), and limb-girdle muscular dystrophies (sarcoglycanopathies), are caused by mutations in the different DGC components. Although many early studies indicated DGC plays a crucial mechanical role in maintaining the structural integrity of skeletal muscle, recent studies identified novel roles of DGC. Beyond a mechanical role, these DGC members play important signaling roles and act as a scaffold for various signaling pathways. For example, neuronal nitric oxide synthase (nNOS), which is localized at the muscle membrane by DGC members (dystrophin and syntrophins), plays an important role in the regulation of the blood flow during exercise. DGC also plays important roles at the neuromuscular junction (NMJ) and in the brain. In this review, we will focus on recently identified roles of DGC particularly in exercise and the brain. |
| first_indexed | 2024-12-23T05:21:39Z |
| format | Article |
| id | doaj.art-d0668b4083bc40009fa60c74b98eaffb |
| institution | Directory Open Access Journal |
| issn | 2076-3425 |
| language | English |
| last_indexed | 2024-12-23T05:21:39Z |
| publishDate | 2015-07-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Brain Sciences |
| spelling | doaj.art-d0668b4083bc40009fa60c74b98eaffb2022-12-21T17:58:42ZengMDPI AGBrain Sciences2076-34252015-07-015327529810.3390/brainsci5030275brainsci5030275Nonmechanical Roles of Dystrophin and Associated Proteins in Exercise, Neuromuscular Junctions, and BrainsBailey Nichols0Shin'ichi Takeda1Toshifumi Yokota2Department of Medical Genetics, University of Alberta Faculty of Medicine and DentistryDepartment of Molecular Therapy, National Institute of Neuroscience, National Center of Neurology and Psychiatry, 4-1-1, Ogawa-higashi, Kodaira, Tokyo 187-8502, JapanDepartment of Medical Genetics, University of Alberta Faculty of Medicine and DentistryDystrophin-glycoprotein complex (DGC) is an important structural unit in skeletal muscle that connects the cytoskeleton (f-actin) of a muscle fiber to the extracellular matrix (ECM). Several muscular dystrophies, such as Duchenne muscular dystrophy, Becker muscular dystrophy, congenital muscular dystrophies (dystroglycanopathies), and limb-girdle muscular dystrophies (sarcoglycanopathies), are caused by mutations in the different DGC components. Although many early studies indicated DGC plays a crucial mechanical role in maintaining the structural integrity of skeletal muscle, recent studies identified novel roles of DGC. Beyond a mechanical role, these DGC members play important signaling roles and act as a scaffold for various signaling pathways. For example, neuronal nitric oxide synthase (nNOS), which is localized at the muscle membrane by DGC members (dystrophin and syntrophins), plays an important role in the regulation of the blood flow during exercise. DGC also plays important roles at the neuromuscular junction (NMJ) and in the brain. In this review, we will focus on recently identified roles of DGC particularly in exercise and the brain.http://www.mdpi.com/2076-3425/5/3/275dystrophindystrophin-glycoprotein complex (DGC)syntrophinexercisebraintadalafil (Cialis®)sildenafil citrate (Viagra®)muscular dystrophyneuronal nitric oxide synthase (nNOS)two-hit hypothesis (two-hit theory) |
| spellingShingle | Bailey Nichols Shin'ichi Takeda Toshifumi Yokota Nonmechanical Roles of Dystrophin and Associated Proteins in Exercise, Neuromuscular Junctions, and Brains Brain Sciences dystrophin dystrophin-glycoprotein complex (DGC) syntrophin exercise brain tadalafil (Cialis®) sildenafil citrate (Viagra®) muscular dystrophy neuronal nitric oxide synthase (nNOS) two-hit hypothesis (two-hit theory) |
| title | Nonmechanical Roles of Dystrophin and Associated Proteins in Exercise, Neuromuscular Junctions, and Brains |
| title_full | Nonmechanical Roles of Dystrophin and Associated Proteins in Exercise, Neuromuscular Junctions, and Brains |
| title_fullStr | Nonmechanical Roles of Dystrophin and Associated Proteins in Exercise, Neuromuscular Junctions, and Brains |
| title_full_unstemmed | Nonmechanical Roles of Dystrophin and Associated Proteins in Exercise, Neuromuscular Junctions, and Brains |
| title_short | Nonmechanical Roles of Dystrophin and Associated Proteins in Exercise, Neuromuscular Junctions, and Brains |
| title_sort | nonmechanical roles of dystrophin and associated proteins in exercise neuromuscular junctions and brains |
| topic | dystrophin dystrophin-glycoprotein complex (DGC) syntrophin exercise brain tadalafil (Cialis®) sildenafil citrate (Viagra®) muscular dystrophy neuronal nitric oxide synthase (nNOS) two-hit hypothesis (two-hit theory) |
| url | http://www.mdpi.com/2076-3425/5/3/275 |
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