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Dystroglycan

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Dystroglycan

Dystroglycan 1 (dystrophin-associated glycoprotein 1)
PDB rendering based on 1u2c.
Available structures
PDB Ortholog search: PDBe, RCSB
Identifiers
Symbols  ; 156DAG; A3a; AGRNR; DAG; MDDGC7; MDDGC9
External IDs GeneCards:
RNA expression pattern
Orthologs
Species Human Mouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)
RefSeq (protein)
Location (UCSC)
PubMed search

Dystroglycan is a protein that in humans is encoded by the DAG1 gene.[1][2][3]

Dystroglycan is one of the dystrophin-associated glycoproteins, which is encoded by a 5.5 kb transcript in Homo sapiens on chromosome 3.[4] There are two exons that are separated by a large intron. The spliced exons code for a protein product that is finally cleaved into two non-covalently associated subunits, [alpha] (N-terminal) and [beta] (C-terminal).

Contents

  • Function 1
  • Expression 2
  • Interactions 3
  • See also 4
  • References 5
  • Further reading 6
  • External links 7

Function

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.

Expression

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

Interactions

Dystroglycan has been shown to interact with FYN,[5] C-src tyrosine kinase,[5] Src,[5] NCK1,[5] Grb2,[6] Caveolin 3[7] and SHC1.[5]

See also

References

  1. ^ Skynner MJ, Gangadharan U, Coulton GR, Mason RM, Nikitopoulou A, Brown SD, Blanco G (Jan 1995). "Genetic mapping of the mouse neuromuscular mutation kyphoscoliosis". Genomics 25 (1): 207–13.  
  2. ^ Ibraghimov-Beskrovnaya O, Ervasti JM, Leveille CJ, Slaughter CA, Sernett SW, Campbell KP (Feb 1992). "Primary structure of dystrophin-associated glycoproteins linking dystrophin to the extracellular matrix". Nature 355 (6362): 696–702.  
  3. ^ "Entrez Gene: DAG1 dystroglycan 1 (dystrophin-associated glycoprotein 1)". 
  4. ^ Spence HJ, Dhillon AS, James M, Winder SJ (May 2004). "Dystroglycan, a scaffold for the ERK-MAP kinase cascade". EMBO Reports 5 (5): 484–9.  
  5. ^ a b c d e Sotgia F, Lee H, Bedford MT, Petrucci T, Sudol M, Lisanti MP (Dec 2001). "Tyrosine phosphorylation of beta-dystroglycan at its WW domain binding motif, PPxY, recruits SH2 domain containing proteins". Biochemistry 40 (48): 14585–92.  
  6. ^ Yang B, Jung D, Motto D, Meyer J, Koretzky G, Campbell KP (May 1995). "SH3 domain-mediated interaction of dystroglycan and Grb2". The Journal of Biological Chemistry 270 (20): 11711–4.  
  7. ^ Sotgia F, Lee JK, Das K, Bedford M, Petrucci TC, Macioce P, Sargiacomo M, Bricarelli FD, Minetti C, Sudol M, Lisanti MP (Dec 2000). "Caveolin-3 directly interacts with the C-terminal tail of beta -dystroglycan. Identification of a central WW-like domain within caveolin family members". The Journal of Biological Chemistry 275 (48): 38048–58.  

Further reading

  • Matsumura K, Tomé FM, Collin H, Azibi K, Chaouch M, Kaplan JC, Fardeau M, Campbell KP (Sep 1992). "Deficiency of the 50K dystrophin-associated glycoprotein in severe childhood autosomal recessive muscular dystrophy". Nature 359 (6393): 320–2.  
  • Apel ED, Roberds SL, Campbell KP, Merlie JP (Jul 1995). "Rapsyn may function as a link between the acetylcholine receptor and the agrin-binding dystrophin-associated glycoprotein complex". Neuron 15 (1): 115–26.  
  • Yang B, Jung D, Motto D, Meyer J, Koretzky G, Campbell KP (May 1995). "SH3 domain-mediated interaction of dystroglycan and Grb2". The Journal of Biological Chemistry 270 (20): 11711–4.  
  • Yamada H, Shimizu T, Tanaka T, Campbell KP, Matsumura K (Sep 1994). "Dystroglycan is a binding protein of laminin and merosin in peripheral nerve". FEBS Letters 352 (1): 49–53.  
  • Gee SH, Montanaro F, Lindenbaum MH, Carbonetto S (Jun 1994). "Dystroglycan-alpha, a dystrophin-associated glycoprotein, is a functional agrin receptor". Cell 77 (5): 675–86.  
  • Ibraghimov-Beskrovnaya O, Milatovich A, Ozcelik T, Yang B, Koepnick K, Francke U, Campbell KP (Oct 1993). "Human dystroglycan: skeletal muscle cDNA, genomic structure, origin of tissue specific isoforms and chromosomal localization". Human Molecular Genetics 2 (10): 1651–7.  
  • Yamada H, Denzer AJ, Hori H, Tanaka T, Anderson LV, Fujita S, Fukuta-Ohi H, Shimizu T, Ruegg MA, Matsumura K (Sep 1996). "Dystroglycan is a dual receptor for agrin and laminin-2 in Schwann cell membrane". The Journal of Biological Chemistry 271 (38): 23418–23.  
  • Tian M, Jacobson C, Gee SH, Campbell KP, Carbonetto S, Jucker M (Dec 1996). "Dystroglycan in the cerebellum is a laminin alpha 2-chain binding protein at the glial-vascular interface and is expressed in Purkinje cells". The European Journal of Neuroscience 8 (12): 2739–47.  
  • Matsumura K, Chiba A, Yamada H, Fukuta-Ohi H, Fujita S, Endo T, Kobata A, Anderson LV, Kanazawa I, Campbell KP, Shimizu T (May 1997). "A role of dystroglycan in schwannoma cell adhesion to laminin". The Journal of Biological Chemistry 272 (21): 13904–10.  
  • Pirozzi G, McConnell SJ, Uveges AJ, Carter JM, Sparks AB, Kay BK, Fowlkes DM (Jun 1997). "Identification of novel human WW domain-containing proteins by cloning of ligand targets". The Journal of Biological Chemistry 272 (23): 14611–6.  
  • Lorenzo LE, Godin AG, Wang F, St-Louis M, Carbonetto S, Wiseman PW, Ribeiro-da-Silva A, De Koninck Y (Jun 2014). "Gephyrin clusters are absent from small diameter primary afferent terminals despite the presence of GABA(A) receptors". The Journal of Neuroscience 34 (24): 8300–17.  
  • Gesemann M, Brancaccio A, Schumacher B, Ruegg MA (Jan 1998). "Agrin is a high-affinity binding protein of dystroglycan in non-muscle tissue". The Journal of Biological Chemistry 273 (1): 600–5.  
  • Rambukkana A, Yamada H, Zanazzi G, Mathus T, Salzer JL, Yurchenco PD, Campbell KP, Fischetti VA (Dec 1998). "Role of alpha-dystroglycan as a Schwann cell receptor for Mycobacterium leprae". Science 282 (5396): 2076–9.  
  • Cao W, Henry MD, Borrow P, Yamada H, Elder JH, Ravkov EV, Nichol ST, Compans RW, Campbell KP, Oldstone MB (Dec 1998). "Identification of alpha-dystroglycan as a receptor for lymphocytic choriomeningitis virus and Lassa fever virus". Science 282 (5396): 2079–81.  
  • Shimizu H, Hosokawa H, Ninomiya H, Miner JH, Masaki T (Apr 1999). "Adhesion of cultured bovine aortic endothelial cells to laminin-1 mediated by dystroglycan". The Journal of Biological Chemistry 274 (17): 11995–2000.  
  • Côté PD, Moukhles H, Lindenbaum M, Carbonetto S (Nov 1999). "Chimaeric mice deficient in dystroglycans develop muscular dystrophy and have disrupted myoneural synapses". Nature Genetics 23 (3): 338–42.  
  • Rentschler S, Linn H, Deininger K, Bedford MT, Espanel X, Sudol M (Apr 1999). "The WW domain of dystrophin requires EF-hands region to interact with beta-dystroglycan". Biological Chemistry 380 (4): 431–42.  
  • Tommasi di Vignano A, Di Zenzo G, Sudol M, Cesareni G, Dente L (Apr 2000). "Contribution of the different modules in the utrophin carboxy-terminal region to the formation and regulation of the DAP complex". FEBS Letters 471 (2-3): 229–34.  
  • James M, Nuttall A, Ilsley JL, Ottersbach K, Tinsley JM, Sudol M, Winder SJ (May 2000). "Adhesion-dependent tyrosine phosphorylation of (beta)-dystroglycan regulates its interaction with utrophin". Journal of Cell Science. 113 ( Pt 10) (10): 1717–26.  
  • Russo K, Di Stasio E, Macchia G, Rosa G, Brancaccio A, Petrucci TC (Jul 2000). "Characterization of the beta-dystroglycan-growth factor receptor 2 (Grb2) interaction". Biochemical and Biophysical Research Communications 274 (1): 93–8.  

External links

  • Dystroglycans at the US National Library of Medicine Medical Subject Headings (MeSH)
  • Overview at sdbonline.org
  • http://www.neuro.wustl.edu/neuromuscular/musdist/dag2.htm#ad
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