dystroglycans

Summary

Summary: Dystrophin-associated proteins that play role in the formation of a transmembrane link between laminin-2 and DYSTROPHIN. Both the alpha and the beta subtypes of dystroglycan originate via POST-TRANSLATIONAL PROTEIN PROCESSING of a single precursor protein.

Top Publications

  1. Cai H, Erdman R, Zweier L, Chen J, Shaw J, Baylor K, et al. The sarcoglycan complex in Schwann cells and its role in myelin stability. Exp Neurol. 2007;205:257-69 pubmed
    ..6 hamsters consistent with reduced myelin stability. Together, these findings suggest an important role of sarcoglycans in the stability of peripheral nerve myelin. ..
  2. Hewitt J. Abnormal glycosylation of dystroglycan in human genetic disease. Biochim Biophys Acta. 2009;1792:853-61 pubmed publisher
  3. McDearmon E, Combs A, Sekiguchi K, Fujiwara H, Ervasti J. Brain alpha-dystroglycan displays unique glycoepitopes and preferential binding to laminin-10/11. FEBS Lett. 2006;580:3381-5 pubmed
    ..Our results suggest that tissue-specific glycosylation modifies the laminin binding specificity of alpha-dystroglycan. ..
  4. Batchelor C, Higginson J, Chen Y, Vanni C, Eva A, Winder S. Recruitment of Dbl by ezrin and dystroglycan drives membrane proximal Cdc42 activation and filopodia formation. Cell Cycle. 2007;6:353-63 pubmed
    ..Depletion of dystroglycan inhibited Cdc42-induced filopodia formation. For the first time we also demonstrate co-localization of Cdc42 and dystroglycan at the tips of dynamic filopodia. ..
  5. Gawlik K, Mayer U, Blomberg K, Sonnenberg A, Ekblom P, Durbeej M. Laminin alpha1 chain mediated reduction of laminin alpha2 chain deficient muscular dystrophy involves integrin alpha7beta1 and dystroglycan. FEBS Lett. 2006;580:1759-65 pubmed
    ..We suggest that LNalpha1 chain in part ameliorates the development of LNalpha2 chain deficient muscular dystrophy by retaining the binding sites for integrin alpha7Bbeta1D and alpha-dystroglycan, respectively. ..
  6. Sciandra F, Bozzi M, Morlacchi S, Galtieri A, Giardina B, Brancaccio A. Mutagenesis at the alpha-beta interface impairs the cleavage of the dystroglycan precursor. FEBS J. 2009;276:4933-45 pubmed publisher
    ..These data reinforce the notion that DG processing and its membrane targeting are two independent processes, and shed new light on the molecular mechanism that drives the maturation of the DG precursor. ..
  7. Schneider M, Khalil A, Poulton J, Castillejo Lopez C, Egger Adam D, Wodarz A, et al. Perlecan and Dystroglycan act at the basal side of the Drosophila follicular epithelium to maintain epithelial organization. Development. 2006;133:3805-15 pubmed
    ..We suggest that the interaction of Pcan and Dg at the basal side of the epithelium promotes basal membrane differentiation and is required for maintenance of cell polarity in the FCE. ..
  8. Rezniczek G, Konieczny P, Nikolic B, Reipert S, Schneller D, Abrahamsberg C, et al. Plectin 1f scaffolding at the sarcolemma of dystrophic (mdx) muscle fibers through multiple interactions with beta-dystroglycan. J Cell Biol. 2007;176:965-77 pubmed
    ..It also explains the plectin phenotype observed in dystrophic skeletal muscle of mdx mice and Duchenne muscular dystrophy patients...
  9. Roscioli T, Kamsteeg E, Buysse K, Maystadt I, van Reeuwijk J, van den Elzen C, et al. Mutations in ISPD cause Walker-Warburg syndrome and defective glycosylation of ?-dystroglycan. Nat Genet. 2012;44:581-5 pubmed publisher
    ..These results implicate ISPD in a-dystroglycan glycosylation in maintaining sarcolemma integrity in vertebrates. ..
  10. Lunardi A, Cremisi F, Dente L. Dystroglycan is required for proper retinal layering. Dev Biol. 2006;290:411-20 pubmed
    ..These results recall the phenotypes observed in specific human diseases and suggest that DG presence is crucial at early stages for the organization of retinal architecture. ..

Detail Information

Publications108 found, 100 shown here

  1. Cai H, Erdman R, Zweier L, Chen J, Shaw J, Baylor K, et al. The sarcoglycan complex in Schwann cells and its role in myelin stability. Exp Neurol. 2007;205:257-69 pubmed
    ..6 hamsters consistent with reduced myelin stability. Together, these findings suggest an important role of sarcoglycans in the stability of peripheral nerve myelin. ..
  2. Hewitt J. Abnormal glycosylation of dystroglycan in human genetic disease. Biochim Biophys Acta. 2009;1792:853-61 pubmed publisher
  3. McDearmon E, Combs A, Sekiguchi K, Fujiwara H, Ervasti J. Brain alpha-dystroglycan displays unique glycoepitopes and preferential binding to laminin-10/11. FEBS Lett. 2006;580:3381-5 pubmed
    ..Our results suggest that tissue-specific glycosylation modifies the laminin binding specificity of alpha-dystroglycan. ..
  4. Batchelor C, Higginson J, Chen Y, Vanni C, Eva A, Winder S. Recruitment of Dbl by ezrin and dystroglycan drives membrane proximal Cdc42 activation and filopodia formation. Cell Cycle. 2007;6:353-63 pubmed
    ..Depletion of dystroglycan inhibited Cdc42-induced filopodia formation. For the first time we also demonstrate co-localization of Cdc42 and dystroglycan at the tips of dynamic filopodia. ..
  5. Gawlik K, Mayer U, Blomberg K, Sonnenberg A, Ekblom P, Durbeej M. Laminin alpha1 chain mediated reduction of laminin alpha2 chain deficient muscular dystrophy involves integrin alpha7beta1 and dystroglycan. FEBS Lett. 2006;580:1759-65 pubmed
    ..We suggest that LNalpha1 chain in part ameliorates the development of LNalpha2 chain deficient muscular dystrophy by retaining the binding sites for integrin alpha7Bbeta1D and alpha-dystroglycan, respectively. ..
  6. Sciandra F, Bozzi M, Morlacchi S, Galtieri A, Giardina B, Brancaccio A. Mutagenesis at the alpha-beta interface impairs the cleavage of the dystroglycan precursor. FEBS J. 2009;276:4933-45 pubmed publisher
    ..These data reinforce the notion that DG processing and its membrane targeting are two independent processes, and shed new light on the molecular mechanism that drives the maturation of the DG precursor. ..
  7. Schneider M, Khalil A, Poulton J, Castillejo Lopez C, Egger Adam D, Wodarz A, et al. Perlecan and Dystroglycan act at the basal side of the Drosophila follicular epithelium to maintain epithelial organization. Development. 2006;133:3805-15 pubmed
    ..We suggest that the interaction of Pcan and Dg at the basal side of the epithelium promotes basal membrane differentiation and is required for maintenance of cell polarity in the FCE. ..
  8. Rezniczek G, Konieczny P, Nikolic B, Reipert S, Schneller D, Abrahamsberg C, et al. Plectin 1f scaffolding at the sarcolemma of dystrophic (mdx) muscle fibers through multiple interactions with beta-dystroglycan. J Cell Biol. 2007;176:965-77 pubmed
    ..It also explains the plectin phenotype observed in dystrophic skeletal muscle of mdx mice and Duchenne muscular dystrophy patients...
  9. Roscioli T, Kamsteeg E, Buysse K, Maystadt I, van Reeuwijk J, van den Elzen C, et al. Mutations in ISPD cause Walker-Warburg syndrome and defective glycosylation of ?-dystroglycan. Nat Genet. 2012;44:581-5 pubmed publisher
    ..These results implicate ISPD in a-dystroglycan glycosylation in maintaining sarcolemma integrity in vertebrates. ..
  10. Lunardi A, Cremisi F, Dente L. Dystroglycan is required for proper retinal layering. Dev Biol. 2006;290:411-20 pubmed
    ..These results recall the phenotypes observed in specific human diseases and suggest that DG presence is crucial at early stages for the organization of retinal architecture. ..
  11. Lefeber D, de Brouwer A, Morava E, Riemersma M, Schuurs Hoeijmakers J, Absmanner B, et al. Autosomal recessive dilated cardiomyopathy due to DOLK mutations results from abnormal dystroglycan O-mannosylation. PLoS Genet. 2011;7:e1002427 pubmed publisher
    ..We thus identified a combined deficiency of protein N-glycosylation and alpha-dystroglycan O-mannosylation in patients with nonsyndromic DCM due to autosomal recessive DOLK mutations. ..
  12. Zhou Y, Thomason D, Gullberg D, Jarrett H. Binding of laminin alpha1-chain LG4-5 domain to alpha-dystroglycan causes tyrosine phosphorylation of syntrophin to initiate Rac1 signaling. Biochemistry. 2006;45:2042-52 pubmed
    ..Syntrophin, tyrosine phosphate, beta-dystroglycan, and Rac1 all co-localize to the sarcolemma of rat muscle sections. A model for how this phosphorylation may initiate downstream events in laminin signaling is presented. ..
  13. Bello V, Sirour C, Moreau N, Denker E, Darribère T. A function for dystroglycan in pronephros development in Xenopus laevis. Dev Biol. 2008;317:106-20 pubmed publisher
    ..They also indicate that Dg may induce a signal transduction pathway controlling cell proliferation needed for the formation of tubules and their growth. ..
  14. 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
    ..These data provide direct mechanistic insight into how the dystroglycan-linked basal lamina contributes to the maintenance of sarcolemmal integrity and protects muscles from damage...
  15. 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
    ..These findings expand our understanding of the mechanisms that underlie congenital muscular dystrophy. ..
  16. Sharma P, Ghavami S, Stelmack G, McNeill K, Mutawe M, Klonisch T, et al. beta-Dystroglycan binds caveolin-1 in smooth muscle: a functional role in caveolae distribution and Ca2+ release. J Cell Sci. 2010;123:3061-70 pubmed publisher
  17. Kucherenko M, Pantoja M, Yatsenko A, Shcherbata H, Fischer K, Maksymiv D, et al. Genetic modifier screens reveal new components that interact with the Drosophila dystroglycan-dystrophin complex. PLoS ONE. 2008;3:e2418 pubmed publisher
    ..These data suggest that the Dg-Dys complex and the other pathways regulating extracellular information transfer to the cytoskeletal dynamics are more intercalated than previously thought. ..
  18. Oppizzi M, Akhavan A, Singh M, Fata J, Muschler J. Nuclear translocation of beta-dystroglycan reveals a distinctive trafficking pattern of autoproteolyzed mucins. Traffic. 2008;9:2063-72 pubmed publisher
    ..They also reveal an evolutionarily conserved role for SEA autoproteolysis, serving to enable independent functions of mucin transmembrane subunits, enacted by a shared and poorly understood pathway of segregated subunit trafficking. ..
  19. Bozzi M, Morlacchi S, Bigotti M, Sciandra F, Brancaccio A. Functional diversity of dystroglycan. Matrix Biol. 2009;28:179-87 pubmed publisher
  20. Hara Y, Balci Hayta B, Yoshida Moriguchi T, Kanagawa M, Beltran Valero de Bernabe D, Gundesli H, et al. A dystroglycan mutation associated with limb-girdle muscular dystrophy. N Engl J Med. 2011;364:939-46 pubmed publisher
  21. Colognato H, Galvin J, Wang Z, Relucio J, Nguyen T, Harrison D, et al. Identification of dystroglycan as a second laminin receptor in oligodendrocytes, with a role in myelination. Development. 2007;134:1723-36 pubmed
  22. Higginson J, Thompson O, Winder S. Targeting of dystroglycan to the cleavage furrow and midbody in cytokinesis. Int J Biochem Cell Biol. 2008;40:892-900 pubmed
    ..Dystroglycan may therefore not only have a role in organising the contractile ring through direct or indirect associations with actin, but can also modulate the cell cycle by affecting extracellular signal-regulated kinase levels...
  23. Murakami T, Hayashi Y, Noguchi S, Ogawa M, Nonaka I, Tanabe Y, et al. Fukutin gene mutations cause dilated cardiomyopathy with minimal muscle weakness. Ann Neurol. 2006;60:597-602 pubmed
    ..FKTN mutations could cause much wider spectrum of clinical features than previously perceived, including familial dilated cardiomyopathy and mildest limb girdle muscular dystrophy. ..
  24. Sgambato A, Caredda E, Leocata P, Rossi G, Boninsegna A, Vitale A, et al. Expression of alpha-dystroglycan correlates with tumour grade and predicts survival in oral squamous cell carcinoma. Pathology. 2010;42:248-54 pubmed publisher
    ..Further studies are warranted on the role of this molecule in the entire multistep process of oral squamous tumorigenesis. ..
  25. Zhang Z, Zhang P, Hu H. LARGE expression augments the glycosylation of glycoproteins in addition to ?-dystroglycan conferring laminin binding. PLoS ONE. 2011;6:e19080 pubmed publisher
    ..These results indicate that overexpression of LARGE catalyzes the glycosylation of at least one other glycoprotein in addition to ?-DG, and that this glycosylation(s) promotes laminin binding activity. ..
  26. 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
    ..These data indicate that, similar to its role in skin, alpha6beta4 integrin confers stability to myelin in peripheral nerves. ..
  27. 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
    ..Dynactin-4 and a subset of microtubules disappear from sarcolemmal sites in ankyrin-B-depleted muscle. Ankyrin-B thus is an adaptor required for sarcolemmal localization of dystrophin, as well as dynactin-4...
  28. Sgambato A, Camerini A, Genovese G, De Luca F, Viacava P, Migaldi M, et al. Loss of nuclear p27(kip1) and ?-dystroglycan is a frequent event and is a strong predictor of poor outcome in renal cell carcinoma. Cancer Sci. 2010;101:2080-6 pubmed publisher
    ..Loss of nuclear p27(kip1) is a frequent event in human RCCs and is a powerful predictor of poor outcome which, in combination with low DG expression, could help to identify high-risk patients with clear cell RCC. ..
  29. Biancheri R, Bertini E, Falace A, Pedemonte M, Rossi A, D Amico A, et al. POMGnT1 mutations in congenital muscular dystrophy: genotype-phenotype correlation and expanded clinical spectrum. Arch Neurol. 2006;63:1491-5 pubmed
    ..Molecular analysis identified 5 mutations, 2 of which are novel. This article adds to what is known about the genotype-phenotype correlation and expands our awareness of the clinical spectrum associated with POMGnT1 mutations. ..
  30. Mercuri E, Messina S, Bruno C, Mora M, Pegoraro E, Comi G, et al. Congenital muscular dystrophies with defective glycosylation of dystroglycan: a population study. Neurology. 2009;72:1802-9 pubmed publisher
    ..Our data broaden the clinical spectrum associated with mutations in glycosyltransferases and provide data on their prevalence in the Italian population. ..
  31. Lefeber D, Schönberger J, Morava E, Guillard M, Huyben K, Verrijp K, et al. Deficiency of Dol-P-Man synthase subunit DPM3 bridges the congenital disorders of glycosylation with the dystroglycanopathies. Am J Hum Genet. 2009;85:76-86 pubmed publisher
    ..This mild Dol-P-Man biosynthesis defect due to DPM3 mutations is a cause for alpha-dystroglycanopathy, thereby bridging the congenital disorders of glycosylation with the dystroglycanopathies. ..
  32. Kucherenko M, Marrone A, Rishko V, Magliarelli H, Shcherbata H. Stress and muscular dystrophy: a genetic screen for dystroglycan and dystrophin interactors in Drosophila identifies cellular stress response components. Dev Biol. 2011;352:228-42 pubmed publisher
  33. Liu J, Ball S, Yang Y, Mei P, Zhang L, Shi H, et al. A genetic model for muscle-eye-brain disease in mice lacking protein O-mannose 1,2-N-acetylglucosaminyltransferase (POMGnT1). Mech Dev. 2006;123:228-40 pubmed
    ..The present study provides the first genetic animal model to further dissect the roles of POMGnT1 in MEB disease. ..
  34. Rurak J, Noel G, Lui L, Joshi B, Moukhles H. Distribution of potassium ion and water permeable channels at perivascular glia in brain and retina of the Large(myd) mouse. J Neurochem. 2007;103:1940-53 pubmed
    ..1 and AQP4 to functional domains in brain, distinct mechanisms may contribute to their localization in retina. ..
  35. Bozzi M, Inzitari R, Sbardell D, Monaco S, Pavoni E, Gioia M, et al. Enzymatic processing of beta-dystroglycan recombinant ectodomain by MMP-9: identification of the main cleavage site. IUBMB Life. 2009;61:1143-52 pubmed publisher
  36. Godfrey C, Foley A, Clement E, Muntoni F. Dystroglycanopathies: coming into focus. Curr Opin Genet Dev. 2011;21:278-85 pubmed publisher
    ..This review will focus on recent studies that have extended our knowledge of the mechanisms underlying dystroglycanopathies and have further characterised this patient population. ..
  37. Sgambato A, Camerini A, Amoroso D, Genovese G, De Luca F, Cecchi M, et al. Expression of dystroglycan correlates with tumor grade and predicts survival in renal cell carcinoma. Cancer Biol Ther. 2007;6:1840-6 pubmed
  38. 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
    ..Taken together, these findings strongly indicate that agrin and alpha-dystroglycan mediate lymphocyte activation. Furthermore, agrin-involved lymphocyte activation is mediated by alpha-dystroglycan. ..
  39. van Reeuwijk J, Maugenre S, van den Elzen C, Verrips A, Bertini E, Muntoni F, et al. The expanding phenotype of POMT1 mutations: from Walker-Warburg syndrome to congenital muscular dystrophy, microcephaly, and mental retardation. Hum Mutat. 2006;27:453-9 pubmed
    ..Our data suggest the existence of a disease spectrum of CMD including brain and eye abnormalities resulting from POMT1 mutations. ..
  40. McKee K, Capizzi S, Yurchenco P. Scaffold-forming and Adhesive Contributions of Synthetic Laminin-binding Proteins to Basement Membrane Assembly. J Biol Chem. 2009;284:8984-94 pubmed publisher
    ..These findings may be relevant for an understanding of the pathogenesis and treatment of laminin deficiency states. ..
  41. Wolburg Buchholz K, Mack A, Steiner E, Pfeiffer F, Engelhardt B, Wolburg H. Loss of astrocyte polarity marks blood-brain barrier impairment during experimental autoimmune encephalomyelitis. Acta Neuropathol. 2009;118:219-33 pubmed publisher
    ..Our data suggest that during EAE, loss of beta-dystroglycan-mediated astrocyte foot process anchoring to the basement membrane leads to loss of polarized AQP4 localization in astrocytic endfeet, and thus to edema formation in EAE. ..
  42. Toda T, Chiyonobu T, Xiong H, Tachikawa M, Kobayashi K, Manya H, et al. Fukutin and alpha-dystroglycanopathies. Acta Myol. 2005;24:60-3 pubmed
    ..All are characterized by hypoglycosylated alpha-dystroglycan. Fukutin's function and the relation with other alpha-dystroglycanopathies are discussed. ..
  43. Harrison D, Hussain S, Combs A, Ervasti J, Yurchenco P, Hohenester E. Crystal structure and cell surface anchorage sites of laminin alpha1LG4-5. J Biol Chem. 2007;282:11573-81 pubmed
    ..The combined analysis of structure and activities reveal differences in LG domain interactions that should enable dissection of biological roles of different laminin ligands. ..
  44. Endo T. Dystroglycan glycosylation and its role in alpha-dystroglycanopathies. Acta Myol. 2007;26:165-70 pubmed
    ..Advances in glycobiology are expected to result in a better understanding and in improved treatments of a class of muscular dystrophies called alpha-dystroglycanopathies. ..
  45. Moon Y, Rha S, Zhang X, Jeung H, Yang W, Kwon O, et al. Increments of alpha-dystroglycan expression in liver metastasis correlate with poor survival in gastric cancer. J Surg Oncol. 2009;100:459-65 pubmed publisher
    ..056). This approach may be used to further understanding of the pathogenesis of liver metastasis from gastric cancer. Further studies are warranted to reveal the mechanisms of alpha-DG dysregulation in liver metastasis. ..
  46. Nakamura N, Stalnaker S, Lyalin D, Lavrova O, Wells L, Panin V. Drosophila Dystroglycan is a target of O-mannosyltransferase activity of two protein O-mannosyltransferases, Rotated Abdomen and Twisted. Glycobiology. 2010;20:381-94 pubmed publisher
  47. Nilsson J, Nilsson J, Larson G, Grahn A. Characterization of site-specific O-glycan structures within the mucin-like domain of alpha-dystroglycan from human skeletal muscle. Glycobiology. 2010;20:1160-9 pubmed publisher
    ..Twenty-five glycopeptides were characterized from human alpha-dystroglycan, which provide insight to the complex in vivo O-glycosylation of alpha-dystroglycan. ..
  48. Devisme L, Bouchet C, Gonzales M, Alanio E, Bazin A, Bessières B, et al. Cobblestone lissencephaly: neuropathological subtypes and correlations with genes of dystroglycanopathies. Brain. 2012;135:469-82 pubmed publisher
    ..This material offers a rich resource for studies on the affected neurodevelopmental processes of cobblestone lissencephaly and on the identification of other responsible gene(s)/pathway(s). ..
  49. Moore C, Winder S. The inside and out of dystroglycan post-translational modification. Neuromuscul Disord. 2012;22:959-65 pubmed publisher
  50. Barone R, Aiello C, Race V, Morava E, Foulquier F, Riemersma M, et al. DPM2-CDG: a muscular dystrophy-dystroglycanopathy syndrome with severe epilepsy. Ann Neurol. 2012;72:550-8 pubmed publisher
    ..There was clinical evidence of a muscular dystrophy-dystroglycanopathy syndrome, supported by deficient O-mannosylation by muscle immunohistochemistry...
  51. Barresi R, Campbell K. Dystroglycan: from biosynthesis to pathogenesis of human disease. J Cell Sci. 2006;119:199-207 pubmed
    ..Our increased understanding of the mechanisms of interaction of dystroglycan with its ligands has become an essential tool in deciphering the biological processes related to the human diseases in which the proteins are implicated. ..
  52. Poulton J, Deng W. Dystroglycan down-regulation links EGF receptor signaling and anterior-posterior polarity formation in the Drosophila oocyte. Proc Natl Acad Sci U S A. 2006;103:12775-80 pubmed
    ..Our data indicate that Dystroglycan links EGFR-induced repression of the anterior follicle cell fate and anterior-posterior polarity formation in the oocyte. ..
  53. Saito F, Masaki T, Saito Y, Nakamura A, Takeda S, Shimizu T, et al. Defective peripheral nerve myelination and neuromuscular junction formation in fukutin-deficient chimeric mice. J Neurochem. 2007;101:1712-22 pubmed
    ..They also suggest that defective glycosylation of alpha-dystroglycan may play a role in the impairment of these processes in the deficiency of fukutin. ..
  54. Rojek J, Spiropoulou C, Campbell K, Kunz S. Old World and clade C New World arenaviruses mimic the molecular mechanism of receptor recognition used by alpha-dystroglycan's host-derived ligands. J Virol. 2007;81:5685-95 pubmed
  55. Haines N, Seabrooke S, Stewart B. Dystroglycan and protein O-mannosyltransferases 1 and 2 are required to maintain integrity of Drosophila larval muscles. Mol Biol Cell. 2007;18:4721-30 pubmed
    ..This study opens the possibility of using Drosophila to investigate muscular dystrophy. ..
  56. Akhavan A, Crivelli S, Singh M, Lingappa V, Muschler J. SEA domain proteolysis determines the functional composition of dystroglycan. FASEB J. 2008;22:612-21 pubmed
    ..We provide a structural model for the cleavage domain that is validated by experimental analysis and discuss this cleavage in the context of mucin protein and SEA domain evolution. ..
  57. Christoforou C, Greer C, Challoner B, Charizanos D, Ray R. The detached locus encodes Drosophila Dystrophin, which acts with other components of the Dystrophin Associated Protein Complex to influence intercellular signalling in developing wing veins. Dev Biol. 2008;313:519-32 pubmed
  58. Nicchia G, Rossi A, Nudel U, Svelto M, Frigeri A. Dystrophin-dependent and -independent AQP4 pools are expressed in the mouse brain. Glia. 2008;56:869-76 pubmed publisher
    ..These data provide a better understanding on the association between AQP4 and the dystrophin-glycoprotein complex in the central nervous system. ..
  59. Moore C, Goh H, Hewitt J. Genes required for functional glycosylation of dystroglycan are conserved in zebrafish. Genomics. 2008;92:159-67 pubmed publisher
    ..These data indicate that the dystroglycan glycosylation pathway is conserved in zebrafish and suggest this organism is likely to be a useful model system for functional studies. ..
  60. Court F, Hewitt J, Davies K, Patton B, Uncini A, Wrabetz L, et al. A laminin-2, dystroglycan, utrophin axis is required for compartmentalization and elongation of myelin segments. J Neurosci. 2009;29:3908-19 pubmed publisher
    ..Other cell types may exploit dystroglycan complexes in similar fashions to create barriers and compartments. ..
  61. 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
    ..For the first time, we also demonstrate unequivocally that beta-dystroglycan is a resident of focal adhesions. ..
  62. Frost A, Bohm S, Sewduth R, Josifova D, Ogilvie C, Izatt L, et al. Heterozygous deletion of a 2-Mb region including the dystroglycan gene in a patient with mild myopathy, facial hypotonia, oral-motor dyspraxia and white matter abnormalities. Eur J Hum Genet. 2010;18:852-5 pubmed publisher
  63. Jiang X, Rieder S, Giese N, Friess H, Michalski C, Kleeff J. Reduced ?-dystroglycan expression correlates with shortened patient survival in pancreatic cancer. J Surg Res. 2011;171:120-6 pubmed publisher
    ..In normal pancreatic tissues, ?- and ?-dystroglycans were mainly expressed on the basolateral cell membrane of acinar and ductal cells, while islet cells showed a ..
  64. Stalnaker S, Hashmi S, Lim J, Aoki K, Porterfield M, Gutierrez Sanchez G, et al. Site mapping and characterization of O-glycan structures on alpha-dystroglycan isolated from rabbit skeletal muscle. J Biol Chem. 2010;285:24882-91 pubmed publisher
    ..The distribution of identified sites of O-mannosylation suggests a limited role for local primary sequence in dictating sites of attachment. ..
  65. Hu Y, Li Z, Wu X, Lu Q. Large induces functional glycans in an O-mannosylation dependent manner and targets GlcNAc terminals on alpha-dystroglycan. PLoS ONE. 2011;6:e16866 pubmed publisher
    ..Thus our results revealed a mechanism by which Large competes with galactosyltransferase to target GlcNAc terminals to induce the functional glycans on ?-DG. ..
  66. Bozzi M, Sciandra F, Ferri L, Torreri P, Pavoni E, Petrucci T, et al. Concerted mutation of Phe residues belonging to the beta-dystroglycan ectodomain strongly inhibits the interaction with alpha-dystroglycan in vitro. FEBS J. 2006;273:4929-43 pubmed
    ..As a preliminary analysis of the possible effects of the aforementioned mutations in vivo, detection through immunofluorescence and western blot of the two dystroglycan subunits was pursued in dystroglycan-transfected 293-Ebna cells. ..
  67. Xiong H, Kobayashi K, Tachikawa M, Manya H, Takeda S, Chiyonobu T, et al. Molecular interaction between fukutin and POMGnT1 in the glycosylation pathway of alpha-dystroglycan. Biochem Biophys Res Commun. 2006;350:935-41 pubmed
    ..From these findings, we propose that fukutin forms a complex with POMGnT1 and may modulate its enzymatic activity. ..
  68. Godfrey C, Escolar D, Brockington M, Clement E, Mein R, Jimenez Mallebrera C, et al. Fukutin gene mutations in steroid-responsive limb girdle muscular dystrophy. Ann Neurol. 2006;60:603-10 pubmed
    ..These findings significantly expand the spectrum of phenotypes associated with fukutin mutations to include this novel form of limb girdle muscular dystrophy that we propose to name LGMD2L. ..
  69. Meinen S, Barzaghi P, Lin S, Lochmuller H, Ruegg M. Linker molecules between laminins and dystroglycan ameliorate laminin-alpha2-deficient muscular dystrophy at all disease stages. J Cell Biol. 2007;176:979-93 pubmed
  70. Warth A. Is alpha-dystroglycan the missing link in the mechanism of enterocyte uptake and translocation of Mycobacterium avium paratuberculosis?. Med Hypotheses. 2008;70:369-74 pubmed
    ..Since there are options to modify dystroglycan this might be a potential new target to prevent or even treat intestinal MAP infections. ..
  71. Wairkar Y, Fradkin L, Noordermeer J, DiAntonio A. Synaptic defects in a Drosophila model of congenital muscular dystrophy. J Neurosci. 2008;28:3781-9 pubmed publisher
    ..These results are consistent with the model that dPOMT1-dependent glycosylation of Dg is necessary for proper synaptic function and raise the possibility that similar synaptic defects occur in the congenital muscular dystrophies. ..
  72. 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
    ..This may also advance our understanding of the molecular mechanisms underlying the retinal electrophysiological abnormalities observed in muscular dystrophy patients. ..
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    ..We propose that epiblast-BM interaction requires CLASP- and Dystroglycan-mediated cortical microtubule anchoring, the disruption of which initiates gastrulation EMT. ..
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    ..This supports the notion that additional environmental and/or genetic factors may contribute to the observed broad spectrum of POMGnT1-associated phenotypes. ..
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    ..Characterization of the POMT1 substrate protein alpha-dystroglycan and POMT in vitro mannosyltransferase activity shows that the severity of the clinical phenotype of the patients analyzed is inversely correlated with POMT activity. ..
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    ..Different expression of ?-DG, but not ?-DG, between primary tumor and normal specimen, correlated with patient survival, implicating a potential marker for gastric cancer prognosis. ..
  78. 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
    ..This new model confirms dystroglycan phosphorylation as an important pathway in the aetiology of DMD and provides novel targets for therapeutic intervention. ..
  79. 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
    ..These experiments demonstrate that Galgt1 and Galgt2 contribute in distinct ways to the expression and function of synaptic ?GalNAc-containing carbohydrates at the NMJ. ..
  80. Halayko A, Stelmack G. The association of caveolae, actin, and the dystrophin-glycoprotein complex: a role in smooth muscle phenotype and function?. Can J Physiol Pharmacol. 2005;83:877-91 pubmed
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    ..These observations suggest that glycan-dependent dystroglycan interactions are required for PN neurons to correctly respond to signals at this important migrational checkpoint. ..
  82. Murakami T, Hayashi Y, Ogawa M, Noguchi S, Campbell K, Togawa M, et al. A novel POMT2 mutation causes mild congenital muscular dystrophy with normal brain MRI. Brain Dev. 2009;31:465-8 pubmed publisher
    ..Presence of small amounts of partly glycosylated alpha-DG may have a role in reducing the clinical symptoms of alpha-dystroglycanopathy. ..
  83. Yoon J, Johnson E, Xu R, Martin L, Martin P, Montanaro F. Comparative proteomic profiling of dystroglycan-associated proteins in wild type, mdx, and Galgt2 transgenic mouse skeletal muscle. J Proteome Res. 2012;11:4413-24 pubmed publisher
    ..These studies identify new dystroglycan-associated proteins that may participate in dystroglycan's roles, both positive and negative, in muscular dystrophy. ..
  84. Esser A, Miller M, Huang Q, Meier M, Beltran Valero de Bernabé D, Stipp C, et al. Loss of LARGE2 disrupts functional glycosylation of α-dystroglycan in prostate cancer. J Biol Chem. 2013;288:2132-42 pubmed publisher
    ..Our results describe a novel molecular mechanism to account for the commonly observed hypoglycosylation of αDG in prostate cancer. ..
  85. Yatsenko A, Gray E, Shcherbata H, Patterson L, Sood V, Kucherenko M, et al. A putative Src homology 3 domain binding motif but not the C-terminal dystrophin WW domain binding motif is required for dystroglycan function in cellular polarity in Drosophila. J Biol Chem. 2007;282:15159-69 pubmed
    ..This suggests that an SH3-containing protein, which has yet to be identified, functionally interacts with Dg. ..
  86. Godfrey C, Clement E, Mein R, Brockington M, Smith J, Talim B, et al. Refining genotype phenotype correlations in muscular dystrophies with defective glycosylation of dystroglycan. Brain. 2007;130:2725-35 pubmed
    ..Mutations in these five glycosyltransferase genes were detected in 34% of patients indicating that, after the exclusion of FKRP, the majority of patients with a dystroglycanopathy harbour mutations in novel genes. ..
  87. Clement E, Mercuri E, Godfrey C, Smith J, Robb S, Kinali M, et al. Brain involvement in muscular dystrophies with defective dystroglycan glycosylation. Ann Neurol. 2008;64:573-82 pubmed publisher
    ..Pontine clefts were visible in some dystroglycanopathy patients. Infratentorial structures were often affected in isolation, highlighting their susceptibility to involvement in these conditions. ..
  88. Gazzerro E, Assereto S, Bonetto A, Sotgia F, Scarfi S, Pistorio A, et al. Therapeutic potential of proteasome inhibition in Duchenne and Becker muscular dystrophies. Am J Pathol. 2010;176:1863-77 pubmed publisher
  89. Zhan Y, Melian N, Pantoja M, Haines N, Ruohola Baker H, Bourque C, et al. Dystroglycan and mitochondrial ribosomal protein L34 regulate differentiation in the Drosophila eye. PLoS ONE. 2010;5:e10488 pubmed publisher
    ..We discuss these findings in view of recent work implicating DG as a regulator of cell metabolism and its genetic interaction with mRpL34, a member of a class of mitochondrial genes essential for normal metabolic function. ..
  90. Sirour C, Hidalgo M, Bello V, Buisson N, Darribère T, Moreau N. Dystroglycan is involved in skin morphogenesis downstream of the Notch signaling pathway. Mol Biol Cell. 2011;22:2957-69 pubmed publisher
    ..Our study showed for the first time in vivo that Dg, in addition to organizing laminin in the ECM, also acts as a key signaling component in the Notch pathway. ..
  91. 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
    ..These findings establish a novel role for glycosylated dystroglycan as a key determinant of axon guidance cue distribution and function in the mammalian nervous system. ..