Gene Symbol: Dag1
Description: dystroglycan 1
Alias: D9Wsu13e, Dp427, Dp71, dystroglycan, dystrophin associated glycoprotein 1
Species: mouse
Products:     Dag1

Top Publications

  1. González Ramírez R, Morales Lázaro S, Tapia Ramírez V, Mornet D, Cisneros B. Nuclear and nuclear envelope localization of dystrophin Dp71 and dystrophin-associated proteins (DAPs) in the C2C12 muscle cells: DAPs nuclear localization is modulated during myogenesis. J Cell Biochem. 2008;105:735-45 pubmed publisher
    ..We demonstrated the presence of Dp71, alpha-sarcoglycan, alpha-dystrobrevin, beta-dystroglycan and alpha-syntrophin not only in plasma membrane but also in the nucleus of muscle cells...
  2. Cohn R, Henry M, Michele D, Barresi R, Saito F, Moore S, et al. Disruption of DAG1 in differentiated skeletal muscle reveals a role for dystroglycan in muscle regeneration. Cell. 2002;110:639-48 pubmed
    Striated muscle-specific disruption of the dystroglycan (DAG1) gene results in loss of the dystrophin-glycoprotein complex in differentiated muscle and a remarkably mild muscular dystrophy with hypertrophy and without tissue fibrosis...
  3. Spence H, Chen Y, Batchelor C, Higginson J, Suila H, Carpen O, et al. Ezrin-dependent regulation of the actin cytoskeleton by beta-dystroglycan. Hum Mol Genet. 2004;13:1657-68 pubmed
    b>Dystroglycan is part of an adhesion receptor complex linking the extracellular matrix to the actin cytoskeleton...
  4. Han R, Kanagawa M, Yoshida Moriguchi T, Rader E, Ng R, Michele D, et al. Basal lamina strengthens cell membrane integrity via the laminin G domain-binding motif of alpha-dystroglycan. Proc Natl Acad Sci U S A. 2009;106:12573-9 pubmed publisher
    ..Skeletal muscle basal lamina is linked to the sarcolemma through transmembrane receptors, including integrins and dystroglycan. The function of dystroglycan relies critically on posttranslational glycosylation, a common target shared by a ..
  5. Gee S, Montanaro F, Lindenbaum M, Carbonetto S. Dystroglycan-alpha, a dystrophin-associated glycoprotein, is a functional agrin receptor. Cell. 1994;77:675-86 pubmed
    ..Structural similarities shared by agrin and laminin suggested that the laminin receptor dystroglycan-alpha, part of a dystrophin-receptor complex, might also bind agrin...
  6. Sato S, Omori Y, Katoh K, Kondo M, Kanagawa M, Miyata K, et al. Pikachurin, a dystroglycan ligand, is essential for photoreceptor ribbon synapse formation. Nat Neurosci. 2008;11:923-31 pubmed publisher
    ..Pikachurin colocalized with both dystrophin and dystroglycan at the ribbon synapses...
  7. Williamson R, Henry M, Daniels K, Hrstka R, Lee J, Sunada Y, et al. Dystroglycan is essential for early embryonic development: disruption of Reichert's membrane in Dag1-null mice. Hum Mol Genet. 1997;6:831-41 pubmed
    b>Dystroglycan is a central component of the dystrophin-glycoprotein complex (DGC), a protein assembly that plays a critical role in a variety of muscular dystrophies...
  8. Sotgia F, Lee J, Das K, Bedford M, Petrucci T, Macioce P, et al. Caveolin-3 directly interacts with the C-terminal tail of beta -dystroglycan. Identification of a central WW-like domain within caveolin family members. J Biol Chem. 2000;275:38048-58 pubmed
    ..Here, we demonstrate that caveolin-3 directly interacts with beta-dystroglycan, an integral membrane component of the dystrophin complex...
  9. Moore S, Saito F, Chen J, Michele D, Henry M, Messing A, et al. Deletion of brain dystroglycan recapitulates aspects of congenital muscular dystrophy. Nature. 2002;418:422-5 pubmed
    ..b>Dystroglycan is a highly glycosylated component of the muscle dystrophin-glycoprotein complex that is also expressed in ..

More Information


  1. Satz J, Barresi R, Durbeej M, Willer T, Turner A, Moore S, et al. Brain and eye malformations resembling Walker-Warburg syndrome are recapitulated in mice by dystroglycan deletion in the epiblast. J Neurosci. 2008;28:10567-75 pubmed publisher
    ..two of the causative genes, form an active enzyme complex in the posttranslational biosynthetic pathway of dystroglycan. Deletion of either Pomt1 or the dystroglycan gene causes early embryonic lethality in mice...
  2. Ibraghimov Beskrovnaya O, Milatovich A, Ozcelik T, Yang B, Koepnick K, Francke U, et al. Human dystroglycan: skeletal muscle cDNA, genomic structure, origin of tissue specific isoforms and chromosomal localization. Hum Mol Genet. 1993;2:1651-7 pubmed
    b>Dystroglycan is a novel laminin binding component of the dystrophin-glycoprotein complex which provides a linkage between the subsarcolemmal cytoskeleton and the extracellular matrix...
  3. Berti C, Bartesaghi L, Ghidinelli M, Zambroni D, Figlia G, Chen Z, et al. Non-redundant function of dystroglycan and ?1 integrins in radial sorting of axons. Development. 2011;138:4025-37 pubmed publisher
    ..By contrast, deletion of the Large and fukutin substrate dystroglycan does not result in radial sorting defects...
  4. Kuga A, Kanagawa M, Sudo A, Chan Y, Tajiri M, Manya H, et al. Absence of post-phosphoryl modification in dystroglycanopathy mouse models and wild-type tissues expressing non-laminin binding form of ?-dystroglycan. J Biol Chem. 2012;287:9560-7 pubmed publisher
    b>Dystroglycan (?-DG) is a membrane-associated glycoprotein that interacts with several extracellular matrix proteins, including laminin and agrin...
  5. Anderson C, Winder S, Borycki A. Dystroglycan protein distribution coincides with basement membranes and muscle differentiation during mouse embryogenesis. Dev Dyn. 2007;236:2627-35 pubmed
    Using immunohistochemistry, we have examined beta-Dystroglycan protein distribution in the mouse embryo at embryonic stages E9.5 to E11.5...
  6. Occhi S, Zambroni D, Del Carro U, Amadio S, Sirkowski E, Scherer S, et al. Both laminin and Schwann cell dystroglycan are necessary for proper clustering of sodium channels at nodes of Ranvier. J Neurosci. 2005;25:9418-27 pubmed
    ..Furthermore, mice lacking Schwann cell dystroglycan have aberrant microvilli and poorly clustered sodium channels...
  7. Court F, Zambroni D, Pavoni E, Colombelli C, Baragli C, Figlia G, et al. MMP2-9 cleavage of dystroglycan alters the size and molecular composition of Schwann cell domains. J Neurosci. 2011;31:12208-17 pubmed publisher
    ..How this occurs is essentially unknown. The dystroglycan-dystrophin complex is required for the function of myelin-forming Schwann cells...
  8. Moll J, Barzaghi P, Lin S, Bezakova G, Lochmuller H, Engvall E, et al. An agrin minigene rescues dystrophic symptoms in a mouse model for congenital muscular dystrophy. Nature. 2001;413:302-7 pubmed
    ..Here we show that this mini-agrin-which binds to basement membrane and to alpha-dystroglycan, a member of the DGC-amends muscle pathology by a mechanism that includes agrin-mediated stabilization of alpha-..
  9. Ohlendieck K, Campbell K. Dystrophin-associated proteins are greatly reduced in skeletal muscle from mdx mice. J Cell Biol. 1991;115:1685-94 pubmed
  10. Côté P, Moukhles H, Lindenbaum M, Carbonetto S. Chimaeric mice deficient in dystroglycans develop muscular dystrophy and have disrupted myoneural synapses. Nat Genet. 1999;23:338-42 pubmed
    ..alpha-Dystroglycan (Dg) binds to laminins in the basement membrane surrounding each myofibre and docks with beta-Dg, a ..
  11. Rentschler S, Linn H, Deininger K, Bedford M, Espanel X, Sudol M. The WW domain of dystrophin requires EF-hands region to interact with beta-dystroglycan. Biol Chem. 1999;380:431-42 pubmed
    ..of the dystrophin-associated complex, for example, between dystrophin and the transmembrane glycoprotein beta-dystroglycan, may lead to muscular dystrophy...
  12. Zhang J, Wang Y, Chu Y, Su L, Gong Y, Zhang R, et al. Agrin is involved in lymphocytes activation that is mediated by alpha-dystroglycan. FASEB J. 2006;20:50-8 pubmed
    ..unlike that found in muscle cells where there is coexpression of muscle-specific kinase (MuSK) and alpha-dystroglycan receptors for agrin, only alpha-dystroglycan could be detected in lymphocytes...
  13. Nodari A, Previtali S, Dati G, Occhi S, Court F, Colombelli C, et al. Alpha6beta4 integrin and dystroglycan cooperate to stabilize the myelin sheath. J Neurosci. 2008;28:6714-9 pubmed publisher
    ..When the laminin receptor dystroglycan is also ablated, major folding abnormalities occur, associated with acute demyelination in some peripheral ..
  14. Saito F, Moore S, Barresi R, Henry M, Messing A, Ross Barta S, et al. Unique role of dystroglycan in peripheral nerve myelination, nodal structure, and sodium channel stabilization. Neuron. 2003;38:747-58 pubmed
    b>Dystroglycan is a central component of the dystrophin-glycoprotein complex implicated in the pathogenesis of several neuromuscular diseases...
  15. Michele D, Barresi R, Kanagawa M, Saito F, Cohn R, Satz J, et al. Post-translational disruption of dystroglycan-ligand interactions in congenital muscular dystrophies. Nature. 2002;418:417-22 pubmed
    ..Here we show, in both MEB and FCMD patients, that alpha-dystroglycan is expressed at the muscle membrane, but similar hypoglycosylation in the diseases directly abolishes binding ..
  16. Matsumura K, Ervasti J, Ohlendieck K, Kahl S, Campbell K. Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle. Nature. 1992;360:588-91 pubmed
    ..Because these muscles show minimal pathological changes, our results could provide a basis for the upregulation of DRP as a potential therapeutic approach. ..
  17. Sotgia F, Bonuccelli G, Bedford M, Brancaccio A, Mayer U, Wilson M, et al. Localization of phospho-beta-dystroglycan (pY892) to an intracellular vesicular compartment in cultured cells and skeletal muscle fibers in vivo. Biochemistry. 2003;42:7110-23 pubmed
    beta-Dystroglycan is a ubiquitously expressed integral membrane protein that undergoes tyrosine phosphorylation in an adhesion-dependent manner...
  18. Spence H, Dhillon A, James M, Winder S. Dystroglycan, a scaffold for the ERK-MAP kinase cascade. EMBO Rep. 2004;5:484-9 pubmed
    b>Dystroglycan is an important cell adhesion receptor linking the actin cytoskeleton, via utrophin and dystrophin, to laminin in the extracellular matrix...
  19. Durbeej M, Larsson E, Ibraghimov Beskrovnaya O, Roberds S, Campbell K, Ekblom P. Non-muscle alpha-dystroglycan is involved in epithelial development. J Cell Biol. 1995;130:79-91 pubmed
    The dystroglycan complex is a transmembrane linkage between the cytoskeleton and the basement membrane in muscle...
  20. Durbeej M, Henry M, Ferletta M, Campbell K, Ekblom P. Distribution of dystroglycan in normal adult mouse tissues. J Histochem Cytochem. 1998;46:449-57 pubmed
    b>Dystroglycan is a cell surface protein which, in muscle, links the extracellular matrix protein laminin-2 to the intracellular cytoskeleton. Dystroglycan also binds laminin-1 and the binding occurs via the E3 fragment of laminin-1...
  21. Ayalon G, Davis J, Scotland P, Bennett V. An ankyrin-based mechanism for functional organization of dystrophin and dystroglycan. Cell. 2008;135:1189-200 pubmed publisher
    beta-dystroglycan (DG) and the dystrophin-glycoprotein complex (DGC) are localized at costameres and neuromuscular junctions in the sarcolemma of skeletal muscle...
  22. Singhal N, Xu R, Martin P. Distinct contributions of Galgt1 and Galgt2 to carbohydrate expression and function at the mouse neuromuscular junction. Mol Cell Neurosci. 2012;51:112-26 pubmed publisher
    ..Proper postsynaptic localization of the CT carbohydrate also required muscle expression of dystroglycan, a known muscle substrate for Galgt2...
  23. Thompson O, Moore C, Hussain S, Kleino I, Peckham M, Hohenester E, et al. Modulation of cell spreading and cell-substrate adhesion dynamics by dystroglycan. J Cell Sci. 2010;123:118-27 pubmed publisher
    b>Dystroglycan is a ubiquitously expressed cell adhesion protein...
  24. Greenberg D, Sunada Y, Campbell K, Yaffe D, Nudel U. Exogenous Dp71 restores the levels of dystrophin associated proteins but does not alleviate muscle damage in mdx mice. Nat Genet. 1994;8:340-4 pubmed
    b>Dp71 is a non-muscle product of the Duchenne muscular dystrophy gene. It consists of the cysteine-rich and C-terminal domains of dystrophin...
  25. Johnson E, Zhang L, Adams M, Phillips A, Freitas M, Froehner S, et al. Proteomic analysis reveals new cardiac-specific dystrophin-associated proteins. PLoS ONE. 2012;7:e43515 pubmed publisher
    ..In addition, our findings support the existence of cardiac-specific functions of dystrophin and may guide studies into early triggers of cardiac disease in Duchenne and Becker muscular dystrophies. ..
  26. Yoshida M, Hama H, Ishikawa Sakurai M, Imamura M, Mizuno Y, Araishi K, et al. Biochemical evidence for association of dystrobrevin with the sarcoglycan-sarcospan complex as a basis for understanding sarcoglycanopathy. Hum Mol Genet. 2000;9:1033-40 pubmed
    ..It is thus considered that the sarcoglycan-sarcospan complex is linked to the signaling protein neuronal nitric oxide synthase via alpha-syntrophin associated with dystrobrevin. ..
  27. Kunz S, Rojek J, Kanagawa M, Spiropoulou C, Barresi R, Campbell K, et al. Posttranslational modification of alpha-dystroglycan, the cellular receptor for arenaviruses, by the glycosyltransferase LARGE is critical for virus binding. J Virol. 2005;79:14282-96 pubmed
    ..virus (LCMV), the human pathogenic Lassa fever virus (LFV), and clade C New World arenaviruses is alpha-dystroglycan (alpha-DG), a cell surface receptor for proteins of the extracellular matrix (ECM)...
  28. Patnaik S, Stanley P. Mouse large can modify complex N- and mucin O-glycans on alpha-dystroglycan to induce laminin binding. J Biol Chem. 2005;280:20851-9 pubmed
    ..a protein with two putative glycosyltransferase domains and is required for the generation of functional alpha-dystroglycan (alpha-DG)...
  29. Schwander M, Shirasaki R, Pfaff S, Muller U. Beta1 integrins in muscle, but not in motor neurons, are required for skeletal muscle innervation. J Neurosci. 2004;24:8181-91 pubmed
    ..We conclude that Itgb1 expression in muscle, but not in motor neurons, is critical for NMJ development. ..
  30. Miller G, Moore C, Terry R, La Riviere T, Mitchell A, Piggott R, et al. Preventing phosphorylation of dystroglycan ameliorates the dystrophic phenotype in mdx mouse. Hum Mol Genet. 2012;21:4508-20 pubmed
    ..Tyrosine phosphorylation of dystroglycan has been identified as a possible signal to promote the proteasomal degradation of the DGC...
  31. Satz J, Ostendorf A, Hou S, Turner A, Kusano H, Lee J, et al. Distinct functions of glial and neuronal dystroglycan in the developing and adult mouse brain. J Neurosci. 2010;30:14560-72 pubmed publisher
    ..genes have been identified that encode known or putative glycosyltransferases in the biosynthetic pathway of dystroglycan. Here we test the effects of brain-specific deletion of dystroglycan, and show distinct functions for neuronal ..
  32. Yoshida Moriguchi T, Yu L, Stalnaker S, Davis S, Kunz S, Madson M, et al. O-mannosyl phosphorylation of alpha-dystroglycan is required for laminin binding. Science. 2010;327:88-92 pubmed publisher
    Alpha-dystroglycan (alpha-DG) is a cell-surface glycoprotein that acts as a receptor for both extracellular matrix proteins containing laminin-G domains and certain arenaviruses...
  33. Tremblay M, Carbonetto S. An extracellular pathway for dystroglycan function in acetylcholine receptor aggregation and laminin deposition in skeletal myotubes. J Biol Chem. 2006;281:13365-73 pubmed
    The dystroglycan (DG) complex is involved in agrin-induced acetylcholine receptor clustering downstream of muscle-specific kinase where it regulates the stability of acetylcholine receptor aggregates as well as assembly of the synaptic ..
  34. Wright K, Lyon K, Leung H, Leahy D, Ma L, Ginty D. Dystroglycan organizes axon guidance cue localization and axonal pathfinding. Neuron. 2012;76:931-44 pubmed publisher
    ..for novel determinants of axon guidance, we identified B3gnt1 and ISPD as required for the glycosylation of dystroglycan in vivo...
  35. Gong Y, Zhang R, Zhang J, Xu L, Zhang F, Xu W, et al. Alpha-dystroglycan is involved in positive selection of thymocytes by participating in immunological synapse formation. FASEB J. 2008;22:1426-39 pubmed publisher
    Alpha-dystroglycan has been proved to be involved in lymphocyte activation by participating in immunological synapse (IS) formation...
  36. Kanagawa M, Saito F, Kunz S, Yoshida Moriguchi T, Barresi R, Kobayashi Y, et al. Molecular recognition by LARGE is essential for expression of functional dystroglycan. Cell. 2004;117:953-64 pubmed
    Reduced ligand binding activity of alpha-dystroglycan is associated with muscle and central nervous system pathogenesis in a growing number of muscular dystrophies...
  37. Li S, Qi Y, McKee K, Liu J, Hsu J, Yurchenco P. Integrin and dystroglycan compensate each other to mediate laminin-dependent basement membrane assembly and epiblast polarization. Matrix Biol. 2017;57-58:272-284 pubmed publisher
    ..The influence of laminin-111 (Lm111) and its integrin and dystroglycan (DG) receptors on epiblast in embryoid bodies (EBs), a model for differentiation of the embryonic plate, was ..
  38. Leu M, Bellmunt E, Schwander M, Farinas I, Brenner H, Muller U. Erbb2 regulates neuromuscular synapse formation and is essential for muscle spindle development. Development. 2003;130:2291-301 pubmed
    ..However, functional spindles never develop. Taken together, our data suggest that Erbb2 signaling regulates the formation of both neuromuscular synapses and muscle spindles. ..
  39. Moukhles H, Carbonetto S. Dystroglycan contributes to the formation of multiple dystrophin-like complexes in brain. J Neurochem. 2001;78:824-34 pubmed
    In muscle, dystrophin anchors a complex of proteins at the cell surface which includes alpha-dystroglycan, beta-dystroglycan, syntrophins and dystrobrevins...
  40. Gurniak C, Chevessier F, Jokwitz M, Jönsson F, Perlas E, Richter H, et al. Severe protein aggregate myopathy in a knockout mouse model points to an essential role of cofilin2 in sarcomeric actin exchange and muscle maintenance. Eur J Cell Biol. 2014;93:252-66 pubmed publisher
    ..Levels of smooth muscle ?-actin were increased and remained high in developing muscles, suggesting that cofilin2 plays a crucial role during the exchange of ?-actin isoforms during the early postnatal remodeling of the sarcomere. ..
  41. Kanagawa M, Omori Y, Sato S, Kobayashi K, Miyagoe Suzuki Y, Takeda S, et al. Post-translational maturation of dystroglycan is necessary for pikachurin binding and ribbon synaptic localization. J Biol Chem. 2010;285:31208-16 pubmed publisher
    Pikachurin, the most recently identified ligand of dystroglycan, plays a crucial role in the formation of the photoreceptor ribbon synapse...
  42. Gupton S, Gertler F. Integrin signaling switches the cytoskeletal and exocytic machinery that drives neuritogenesis. Dev Cell. 2010;18:725-36 pubmed publisher
    ..These regulated changes and coordination of cytoskeletal and exocytic machinery may be used in other physiological contexts involving cell motility and morphogenesis. ..
  43. Dubey M, Bugiani M, Ridder M, Postma N, Brouwers E, Polder E, et al. Mice with megalencephalic leukoencephalopathy with cysts: a developmental angle. Ann Neurol. 2015;77:114-31 pubmed publisher
    ..MLC1 expression affects expression of GlialCAM and ClC-2. Abnormal interplay between these proteins is part of the pathomechanisms of MLC. ..
  44. Imperiali M, Thoma C, Pavoni E, Brancaccio A, Callewaert N, Oxenius A. O Mannosylation of alpha-dystroglycan is essential for lymphocytic choriomeningitis virus receptor function. J Virol. 2005;79:14297-308 pubmed
    Alpha-dystroglycan (alpha-DG) was identified as a common receptor for lymphocytic choriomeningitis virus (LCMV) and several other arenaviruses including the human pathogenic Lassa fever virus...
  45. Bunnell T, Jaeger M, Fitzsimons D, Prins K, Ervasti J. Destabilization of the dystrophin-glycoprotein complex without functional deficits in alpha-dystrobrevin null muscle. PLoS ONE. 2008;3:e2604 pubmed publisher
    ..Here we show that the biochemical association between dystrophin and beta-dystroglycan is compromised in adbn(-/-) skeletal muscle, suggesting that alpha-dystrobrevin plays a structural role in ..
  46. Madhavan R, Jarrett H. Interactions between dystrophin glycoprotein complex proteins. Biochemistry. 1995;34:12204-9 pubmed
    ..The interactions identified here were used to refine the existing model of DGC organization to make it consistent with the current data. ..
  47. Ackroyd M, Whitmore C, Prior S, Kaluarachchi M, Nikolic M, Mayer U, et al. Fukutin-related protein alters the deposition of laminin in the eye and brain. J Neurosci. 2011;31:12927-35 pubmed publisher
    ..The defining feature of this group of disorders is the hypoglycosylation of ?-dystroglycan and its inability to effectively bind extracellular matrix ligands such as laminin ?2...
  48. Noell S, Wolburg Buchholz K, Mack A, Beedle A, Satz J, Campbell K, et al. Evidence for a role of dystroglycan regulating the membrane architecture of astroglial endfeet. Eur J Neurosci. 2011;33:2179-86 pubmed publisher
    The dystrophin-dystroglycan complex (DDC) is a molecular array of proteins in muscle and brain cells. The central component of the DDC is dystroglycan, which comprises ?- and ?-subunits...
  49. Willer T, Prados B, Falcón Pérez J, Renner Müller I, Przemeck G, Lommel M, et al. Targeted disruption of the Walker-Warburg syndrome gene Pomt1 in mouse results in embryonic lethality. Proc Natl Acad Sci U S A. 2004;101:14126-31 pubmed
    ..The failure of this membrane to form appears to be the result of abnormal glycosylation and maturation of dystroglycan that may impair recruitment of laminin, a structural component required for the formation of Reichert's ..
  50. Chan Y, Bonnemann C, Lidov H, Kunkel L. Molecular organization of sarcoglycan complex in mouse myotubes in culture. J Cell Biol. 1998;143:2033-44 pubmed
    ..delta-sarcoglycan are in close proximity to one another and that delta-sarcoglycan can be cross-linked to the dystroglycan complex...
  51. Qu Q, Smith F. Alpha-dystroglycan interactions affect cerebellar granule neuron migration. J Neurosci Res. 2004;76:771-82 pubmed
    The interaction of alpha-dystroglycan (a-DG) with its extracellular binding partners requires glycans attached to its mucin core domain, and defects in the glycosylation of a-DG are associated with both muscular dystrophy and neuronal ..
  52. Brancaccio A, Jeno P, Engel J. A single disulfide bridge (Cys182-Cys264) is crucial for alpha-dystroglycan N-terminal domain stability. Ann N Y Acad Sci. 1998;857:228-31 pubmed
  53. Bozzi M, Bianchi M, Sciandra F, Paci M, Giardina B, Brancaccio A, et al. Structural characterization by NMR of the natively unfolded extracellular domain of beta-dystroglycan: toward the identification of the binding epitope for alpha-dystroglycan. Biochemistry. 2003;42:13717-24 pubmed
    b>Dystroglycan (DG) is an adhesion molecule playing a crucial role for tissue stability during both early embriogenesis and adulthood and is composed by two tightly interacting subunits: alpha-DG, membrane-associated and highly glycosylated,..
  54. Dowling P, Doran P, Ohlendieck K. Drastic reduction of sarcalumenin in Dp427 (dystrophin of 427 kDa)-deficient fibres indicates that abnormal calcium handling plays a key role in muscular dystrophy. Biochem J. 2004;379:479-88 pubmed
    ..This confirms the idea that abnormal Ca2+ cycling is involved in Ca2+-induced myonecrosis. Hence, manipulating disturbed Ca2+ handling might represent new modes of abolishing proteolytic degradation in muscular dystrophy. ..
  55. Liou L, Walsh K, Vartanian A, Beltran Valero de Bernabe D, Welch M, Campbell K, et al. Functional glycosylation of dystroglycan is crucial for thymocyte development in the mouse. PLoS ONE. 2010;5:e9915 pubmed publisher
    Alpha-dystroglycan (alpha-DG) is a cell surface receptor providing a molecular link between the extracellular matrix (ECM) and the actin-based cytoskeleton...
  56. Foltz S, Modi J, Melick G, Abousaud M, Luan J, Fortunato M, et al. Abnormal Skeletal Muscle Regeneration plus Mild Alterations in Mature Fiber Type Specification in Fktn-Deficient Dystroglycanopathy Muscular Dystrophy Mice. PLoS ONE. 2016;11:e0147049 pubmed publisher
    Glycosylated α-dystroglycan provides an essential link between extracellular matrix proteins, like laminin, and the cellular cytoskeleton via the dystrophin-glycoprotein complex...
  57. Heller B, Ghidinelli M, Voelkl J, Einheber S, Smith R, Grund E, et al. Functionally distinct PI 3-kinase pathways regulate myelination in the peripheral nervous system. J Cell Biol. 2014;204:1219-36 pubmed publisher
    ..These results identify functionally and spatially distinct PI 3-K pathways: an early, pro-myelinating pathway driven by axonal Neuregulin1 and a later-acting, laminin-integrin-dependent pathway that negatively regulates myelination. ..
  58. Tran D, Lim J, Liu M, Stalnaker S, Wells L, Ten Hagen K, et al. Glycosylation of ?-dystroglycan: O-mannosylation influences the subsequent addition of GalNAc by UDP-GalNAc polypeptide N-acetylgalactosaminyltransferases. J Biol Chem. 2012;287:20967-74 pubmed publisher
    ..b>Dystroglycan (?-DG), a protein linked to the extracellular matrix, whose glycosylation status is associated with human ..
  59. Michele D, Kabaeva Z, Davis S, Weiss R, Campbell K. Dystroglycan matrix receptor function in cardiac myocytes is important for limiting activity-induced myocardial damage. Circ Res. 2009;105:984-93 pubmed publisher
    Genetic mutations in a number of putative glycosyltransferases lead to the loss of glycosylation of dystroglycan and loss of its laminin-binding activity in genetic forms of human muscular dystrophy...
  60. Omori Y, Araki F, Chaya T, Kajimura N, Irie S, Terada K, et al. Presynaptic dystroglycan-pikachurin complex regulates the proper synaptic connection between retinal photoreceptor and bipolar cells. J Neurosci. 2012;32:6126-37 pubmed publisher
    b>Dystroglycan (DG) is a key component of the dystrophin-glycoprotein complex (DGC) at the neuromuscular junction postsynapse...
  61. Heaney D, Schulte B. Dystroglycan expression in the mouse cochlea. Hear Res. 2003;177:12-20 pubmed
    ..Atrophy of strial capillaries in the aging gerbil has been associated with changes in the expression of dystroglycan (DG), a cell-surface receptor that regulates BM assembly...
  62. Straub V, Ettinger A, Durbeej M, Venzke D, Cutshall S, Sanes J, et al. epsilon-sarcoglycan replaces alpha-sarcoglycan in smooth muscle to form a unique dystrophin-glycoprotein complex. J Biol Chem. 1999;274:27989-96 pubmed
    ..Our results also suggest a molecular basis for possible differential smooth muscle dysfunction in sarcoglycan-deficient patients. ..
  63. Munoz J, Zhou Y, Jarrett H. LG4-5 domains of laminin-211 binds alpha-dystroglycan to allow myotube attachment and prevent anoikis. J Cell Physiol. 2010;222:111-9 pubmed publisher
    ..the short laminin globular (LG4-5) modules of the laminin-alpha2 chain C-terminus (called 2E3) that binds alpha-dystroglycan diminishes both apoptosis and necrosis and increases viability while bovine serum albumin had a much lesser ..
  64. Bozic D, Sciandra F, Lamba D, Brancaccio A. The structure of the N-terminal region of murine skeletal muscle alpha-dystroglycan discloses a modular architecture. J Biol Chem. 2004;279:44812-6 pubmed
    b>Dystroglycan (DG) is a cell surface receptor consisting of two subunits: alpha-dystroglycan, extracellular and highly glycosylated, and beta-dystroglycan, spanning the cell membrane...
  65. Ohtsuka Tsurumi E, Saito Y, Yamamoto T, Voit T, Kobayashi M, Osawa M. Co-localization of fukutin and alpha-dystroglycan in the mouse central nervous system. Brain Res Dev Brain Res. 2004;152:121-7 pubmed
    Hypoglycosylation of alpha-dystroglycan (alpha-DG) has been identified in several human diseases associated with muscular dystrophy and brain malformations, including Fukuyama-type congenital muscular dystrophy (FCMD) caused by mutations ..
  66. Kanagawa M, Michele D, Satz J, Barresi R, Kusano H, Sasaki T, et al. Disruption of perlecan binding and matrix assembly by post-translational or genetic disruption of dystroglycan function. FEBS Lett. 2005;579:4792-6 pubmed
    b>Dystroglycan is a cell-surface matrix receptor that requires LARGE-dependent glycosylation for laminin binding...
  67. Song K, Scherer P, Tang Z, Okamoto T, Li S, Chafel M, et al. Expression of caveolin-3 in skeletal, cardiac, and smooth muscle cells. Caveolin-3 is a component of the sarcolemma and co-fractionates with dystrophin and dystrophin-associated glycoproteins. J Biol Chem. 1996;271:15160-5 pubmed
    ..and Src-like kinases) and members of the dystrophin complex (dystrophin, alpha-sarcoglycan, and beta-dystroglycan), but is clearly separated from the bulk of cellular proteins...
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    b>Dystroglycan (DG), a cell adhesion molecule well known to be essential for skeletal muscle integrity and formation of neuromuscular synapses, is also present at inhibitory synapses in the central nervous system...
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    ..was completely abolished in a dose-dependent manner by blocking Ln-1 binding to its alpha(6) integrin and alpha-dystroglycan receptors or by blocking the Ln-1 signaling molecules, phosphatidyl-inositol-3-kinase (P13K) and MAP kinase ..
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    ..We used immunocytochemistry against AQP4, zonula occludens protein-1, glial fibrillary acidic protein, and ?-dystroglycan to follow astrocyte development in E15 to P3 NMRI mouse brains, and expression of AQP4...