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Gene Review

Dag1  -  dystroglycan 1

Mus musculus

Synonyms: D9Wsu13e, DG, Dag-1, Dp427, Dp71, ...
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Disease relevance of Dag1


Psychiatry related information on Dag1

  • We studied 36 patients with muscular dystrophy and either mental retardation, structural brain changes or abnormal alpha-dystroglycan immunolabelling, unlinked to any reported CMD loci [6].
  • Pretreatment with DG (50, 100 and 200 mg/kg p.o.) for seven successive days significantly improved learning and memory in mice and reversed the amnesia induced by both, scopolamine (0.4 mg/kg, i.p.) and natural ageing [7].

High impact information on Dag1


Chemical compound and disease context of Dag1


Biological context of Dag1


Anatomical context of Dag1

  • Overall, the pattern of Drp2 expression corresponds to a subset of the brain regions known to express Dag1, and shows substantial overlap with regions that express various isoforms of dystrophin (particularly in the cerebral cortex, hippocampus and cerebellum) [18].
  • Dp71 was associated with the sarcolemma membrane, where it restored normal expression and localization of all members of the dystrophin-associated glycoprotein complex [19].
  • Dystroglycan-dystrophin complexes are believed to have structural and signaling functions by linking extracellular matrix proteins to the cytoskeleton and cortical signaling molecules [20].
  • Here we characterize a dystroglycan-dystrophin-related protein 2 (DRP2) complex at the surface of myelin-forming Schwann cells [20].
  • Hence, the DRP2-dystroglycan complex likely has a distinct function in the terminal stages of PNS myelinogenesis, possibly in the regulation of myelin thickness [20].

Associations of Dag1 with chemical compounds


Physical interactions of Dag1

  • Therefore, dystroglycan overexpression does not cause the concomitant overexpression of a utrophin-glycoprotein complex in mdx muscles and has no effect on the development of muscle pathology associated with muscular dystrophy [2].
  • Blot overlay assays have shown that perlecan binds alpha-DG in a calcium and heparin-sensitive manner [24].
  • Disruption of the DRP2-dystroglycan complex is followed by hypermyelination and destabilization of the Schwann cell-axon unit in Prx(-/-) mice [20].
  • Biochemical analysis demonstrates that the 120 kDa peripheral nerve alpha-dystroglycan binds merosin as well as laminin [25].
  • In S27 cells, which do not aggregate AChRs spontaneously, agrin and laminin binding to dystroglycan-alpha are dramatically decreased [26].

Enzymatic interactions of Dag1


Co-localisations of Dag1


Regulatory relationships of Dag1


Other interactions of Dag1


Analytical, diagnostic and therapeutic context of Dag1


  1. Targeted inactivation of dystrophin gene product Dp71: phenotypic impact in mouse retina. Dalloz, C., Sarig, R., Fort, P., Yaffe, D., Bordais, A., Pannicke, T., Grosche, J., Mornet, D., Reichenbach, A., Sahel, J., Nudel, U., Rendon, A. Hum. Mol. Genet. (2003) [Pubmed]
  2. Transgenic overexpression of dystroglycan does not inhibit muscular dystrophy in mdx mice. Hoyte, K., Jayasinha, V., Xia, B., Martin, P.T. Am. J. Pathol. (2004) [Pubmed]
  3. Dissociation of the dystroglycan complex in caveolin-3-deficient limb girdle muscular dystrophy. Herrmann, R., Straub, V., Blank, M., Kutzick, C., Franke, N., Jacob, E.N., Lenard, H.G., Kröger, S., Voit, T. Hum. Mol. Genet. (2000) [Pubmed]
  4. Disruption of DAG1 in differentiated skeletal muscle reveals a role for dystroglycan in muscle regeneration. Cohn, R.D., Henry, M.D., Michele, D.E., Barresi, R., Saito, F., Moore, S.A., Flanagan, J.D., Skwarchuk, M.W., Robbins, M.E., Mendell, J.R., Williamson, R.A., Campbell, K.P. Cell (2002) [Pubmed]
  5. The rapid decrease in astrocyte-associated dystroglycan expression by focal cerebral ischemia is protease-dependent. Milner, R., Hung, S., Wang, X., Spatz, M., del Zoppo, G.J. J. Cereb. Blood Flow Metab. (2008) [Pubmed]
  6. Mutations in the human LARGE gene cause MDC1D, a novel form of congenital muscular dystrophy with severe mental retardation and abnormal glycosylation of alpha-dystroglycan. Longman, C., Brockington, M., Torelli, S., Jimenez-Mallebrera, C., Kennedy, C., Khalil, N., Feng, L., Saran, R.K., Voit, T., Merlini, L., Sewry, C.A., Brown, S.C., Muntoni, F. Hum. Mol. Genet. (2003) [Pubmed]
  7. Antiamnesic effects of Desmodium gangeticum in mice. Joshi, H., Parle, M. Yakugaku Zasshi (2006) [Pubmed]
  8. Molecular recognition by LARGE is essential for expression of functional dystroglycan. Kanagawa, M., Saito, F., Kunz, S., Yoshida-Moriguchi, T., Barresi, R., Kobayashi, Y.M., Muschler, J., Dumanski, J.P., Michele, D.E., Oldstone, M.B., Campbell, K.P. Cell (2004) [Pubmed]
  9. Mutant glycosyltransferase and altered glycosylation of alpha-dystroglycan in the myodystrophy mouse. Grewal, P.K., Holzfeind, P.J., Bittner, R.E., Hewitt, J.E. Nat. Genet. (2001) [Pubmed]
  10. Deficiency of a glycoprotein component of the dystrophin complex in dystrophic muscle. Ervasti, J.M., Ohlendieck, K., Kahl, S.D., Gaver, M.G., Campbell, K.P. Nature (1990) [Pubmed]
  11. Pharmacological rescue of the dystrophin-glycoprotein complex in Duchenne and Becker skeletal muscle explants by proteasome inhibitor treatment. Assereto, S., Stringara, S., Sotgia, F., Bonuccelli, G., Broccolini, A., Pedemonte, M., Traverso, M., Biancheri, R., Zara, F., Bruno, C., Lisanti, M.P., Minetti, C. Am. J. Physiol., Cell Physiol. (2006) [Pubmed]
  12. Molecular analysis of the interaction of LCMV with its cellular receptor [alpha]-dystroglycan. Kunz, S., Sevilla, N., McGavern, D.B., Campbell, K.P., Oldstone, M.B. J. Cell Biol. (2001) [Pubmed]
  13. Altered Gene Expression Related to Glomerulogenesis and Podocyte Structure in Early Diabetic Nephropathy of db/db Mice and Its Restoration by Pioglitazone. Makino, H., Miyamoto, Y., Sawai, K., Mori, K., Mukoyama, M., Nakao, K., Yoshimasa, Y., Suga, S. Diabetes (2006) [Pubmed]
  14. Initiating activity of eight pyrolysates of carbohydrates in a two-stage mouse skin tumorigenesis model. Miyakawa, Y., Nishi, Y., Kato, K., Sato, H., Takahashi, M., Hayashi, Y. Carcinogenesis (1991) [Pubmed]
  15. Dystroglycan: brain localisation and chromosome mapping in the mouse. Górecki, D.C., Derry, J.M., Barnard, E.A. Hum. Mol. Genet. (1994) [Pubmed]
  16. Localization of phospho-beta-dystroglycan (pY892) to an intracellular vesicular compartment in cultured cells and skeletal muscle fibers in vivo. Sotgia, F., Bonuccelli, G., Bedford, M., Brancaccio, A., Mayer, U., Wilson, M.T., Campos-Gonzalez, R., Brooks, J.W., Sudol, M., Lisanti, M.P. Biochemistry (2003) [Pubmed]
  17. Identification of the beta-dystroglycan binding epitope within the C-terminal region of alpha-dystroglycan. Sciandra, F., Schneider, M., Giardina, B., Baumgartner, S., Petrucci, T.C., Brancaccio, A. Eur. J. Biochem. (2001) [Pubmed]
  18. Expression of the dystrophin-related protein 2 (Drp2) transcript in the mouse. Dixon, A.K., Tait, T.M., Campbell, E.A., Bobrow, M., Roberts, R.G., Freeman, T.C. J. Mol. Biol. (1997) [Pubmed]
  19. Dp71 can restore the dystrophin-associated glycoprotein complex in muscle but fails to prevent dystrophy. Cox, G.A., Sunada, Y., Campbell, K.P., Chamberlain, J.S. Nat. Genet. (1994) [Pubmed]
  20. Specific disruption of a schwann cell dystrophin-related protein complex in a demyelinating neuropathy. Sherman, D.L., Fabrizi, C., Gillespie, C.S., Brophy, P.J. Neuron (2001) [Pubmed]
  21. Dystroglycan is not required for localization of dystrophin, syntrophin, and neuronal nitric-oxide synthase at the sarcolemma but regulates integrin alpha 7B expression and caveolin-3 distribution. Côté, P.D., Moukhles, H., Carbonetto, S. J. Biol. Chem. (2002) [Pubmed]
  22. Selective reduction in the nicotinic acetylcholine receptor and dystroglycan at the postsynaptic apparatus of mdx mouse superior cervical ganglion. Zaccaria, M.L., De Stefano, M.E., Gotti, C., Petrucci, T.C., Paggi, P. J. Neuropathol. Exp. Neurol. (2000) [Pubmed]
  23. Utrophin-dystroglycan complex in membranes of adherent cultured cells. James, M., Nguyen, T.M., Wise, C.J., Jones, G.E., Morris, G.E. Cell Motil. Cytoskeleton (1996) [Pubmed]
  24. The relationship between perlecan and dystroglycan and its implication in the formation of the neuromuscular junction. Peng, H.B., Ali, A.A., Daggett, D.F., Rauvala, H., Hassell, J.R., Smalheiser, N.R. Cell Adhes. Commun. (1998) [Pubmed]
  25. Dystroglycan is a binding protein of laminin and merosin in peripheral nerve. Yamada, H., Shimizu, T., Tanaka, T., Campbell, K.P., Matsumura, K. FEBS Lett. (1994) [Pubmed]
  26. Dystroglycan-alpha, a dystrophin-associated glycoprotein, is a functional agrin receptor. Gee, S.H., Montanaro, F., Lindenbaum, M.H., Carbonetto, S. Cell (1994) [Pubmed]
  27. Effect of beta-dystroglycan processing on utrophin/Dp116 anchorage in normal and mdx mouse Schwann cell membrane. Hnia, K., Hugon, G., Masmoudi, A., Mercier, J., Rivier, F., Mornet, D. Neuroscience (2006) [Pubmed]
  28. Agrin is involved in lymphocytes activation that is mediated by alpha-dystroglycan. Zhang, J., Wang, Y., Chu, Y., Su, L., Gong, Y., Zhang, R., Xiong, S. FASEB J. (2006) [Pubmed]
  29. The cellular prion protein colocalizes with the dystroglycan complex in the brain. Keshet, G.I., Bar-Peled, O., Yaffe, D., Nudel, U., Gabizon, R. J. Neurochem. (2000) [Pubmed]
  30. G-utrophin, the autosomal homologue of dystrophin Dp116, is expressed in sensory ganglia and brain. Blake, D.J., Schofield, J.N., Zuellig, R.A., Górecki, D.C., Phelps, S.R., Barnard, E.A., Edwards, Y.H., Davies, K.E. Proc. Natl. Acad. Sci. U.S.A. (1995) [Pubmed]
  31. Caveolin-3 directly interacts with the C-terminal tail of beta -dystroglycan. Identification of a central WW-like domain within caveolin family members. Sotgia, F., Lee, J.K., Das, K., Bedford, M., Petrucci, T.C., Macioce, P., Sargiacomo, M., Bricarelli, F.D., Minetti, C., Sudol, M., Lisanti, M.P. J. Biol. Chem. (2000) [Pubmed]
  32. Biochemical characterization of the epithelial dystroglycan complex. Durbeej, M., Campbell, K.P. J. Biol. Chem. (1999) [Pubmed]
  33. Utrophin and dystrophin-associated glycoproteins in normal and dystrophin deficient cardiac muscle. Rivier, F., Robert, A., Royuela, M., Hugon, G., Bonet-Kerrache, A., Mornet, D. J. Muscle Res. Cell. Motil. (1999) [Pubmed]
  34. Transient expression of Dp140, a product of the Duchenne muscular dystrophy locus, during kidney tubulogenesis. Durbeej, M., Jung, D., Hjalt, T., Campbell, K.P., Ekblom, P. Dev. Biol. (1997) [Pubmed]
  35. Dystroglycan distribution in adult mouse brain: a light and electron microscopy study. Zaccaria, M.L., Di Tommaso, F., Brancaccio, A., Paggi, P., Petrucci, T.C. Neuroscience (2001) [Pubmed]
  36. Dystroglycan expression in the wild type and mdx mouse neural retina: synaptic colocalization with dystrophin, dystrophin-related protein but not laminin. Montanaro, F., Carbonetto, S., Campbell, K.P., Lindenbaum, M. J. Neurosci. Res. (1995) [Pubmed]
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