Dystroglycan
You don't need to be Editor-In-Chief to add or edit content to WikiDoc. You can begin to add to or edit text on this WikiDoc page by clicking on the edit button at the top of this page. Next enter or edit the information that you would like to appear here. Once you are done editing, scroll down and click the Save page button at the bottom of the page.
| dystroglycan 1 (dystrophin-associated glycoprotein 1)
| |
| Identifiers | |
| Symbol | DAG1 |
| Entrez | 1605 |
| HUGO | 2666 |
| OMIM | 128239 |
| RefSeq | NM_004393 |
| UniProt | Q14118 |
| Other data | |
| Locus | Chr. 3 p21 |
Dystroglycan is one of the dystrophin-associated glycoproteins, which is encoded by a 5.5 kb transcript in Homo sapiens by chromosome 3. There are two exons that are separated by a large intron. The spliced exons codes for a protein product is finally cleaved into two non-covalently associated subunits, [alpha] (N-terminal) and [beta] (C-terminal). In skeletal muscle the dystroglycan complex works as a transmembrane linkage between the extracellular matrix and the cytoskeleton. [alpha]-dystroglycan is extracellular and binds to merosin [[[alpha]-2 laminin]] in the basement membrane, while [beta]-dystroglycan is a transmembrane protein and binds to dystrophin, which is a large rod-like cytoskeletal protein, absent in Duchenne muscular dystrophy patients. Dystrophin binds to intracellular actin cables. In this way, the dystroglycan complex, which links the extracellular matrix to the intracellular actin cables, is thought to provide structural integrity in muscle tissues. The dystroglycan complex is also known to serve as an agrin receptor in muscle, where it may regulate agrin-induced acetylcholine receptor clustering at the neuromuscular junction. There is also evidence which suggests the function of dystroglycan as a part of the signal transduction pathway because it is shown that Grb2, a mediator of the Ras-related signal pathway, can interact with the cytoplasmic domain of dystroglycan. In general, aberrant expression of dystrophin-associated protein complex underlies the pathogenesis of Duchenne muscular dystrophy, Becker muscular dystrophy and severe childhood autosomal recessive muscular dystrophy. Interestingly, no genetic disease has been described for either [alpha]- or [beta]-dystroglycan. Dystroglycan is widely distributed in non-muscle tissues as well as in muscle tissues. During epithelial morphogenesis of kidney, the dystroglycan complex is shown to act as a receptor for the basement membrane. Dystroglycan expression in Mus musculus brain and neural retina has also been reported. However, the physiological role of dystroglycan in non-muscle tissues has remained unclear
See also
External links
- MeSH Dystroglycans
- Overview at sdbonline.org
- http://www.neuro.wustl.edu/neuromuscular/musdist/dag2.htm#ad
- http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1299052
- http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=128239
 | This cell biology article is a stub. You can help WikiDoc by expanding it. |
Proteins of the cytoskeleton | |
|---|---|
| Microfilaments | Actins - Actin-binding proteins - Actinin - Arp2/3 complex - Cofilin - Destrin - Gelsolin - Myosins - Profilin - Tropomodulin - Troponin (T, C, I) - Tropomyosin - Wiskott-Aldrich syndrome protein |
| Intermediate filaments | type 1 and 2 (Cytokeratin, type I, type II) - type 3 (Desmin, GFAP, Peripherin, Vimentin) - type 4 (Internexin, Nestin, Neurofilament, Synemin, Syncoilin) - type 5 (Lamin A, B) |
| Microtubules | Dyneins - Kinesins - MAPs (Tau protein, Dynamin) - Tubulins - Stathmin - Tektin |
| Catenins | Alpha catenin - Beta catenin - Plakoglobin (gamma catenin) - Delta catenin |
| Nonhuman | Major sperm proteins - Prokaryotic cytoskeleton (Crescentin, FtsZ, MreB) |
| Other | APC - Dystrophin (Dystroglycan) - plakin (Desmoplakin, Plectin) - Spectrin - Talin - Utrophin - Vinculin |
