dystrophin associated protein complex


Summary: A macromolecular complex of proteins that includes DYSTROPHIN and DYSTROPHIN-ASSOCIATED PROTEINS. It plays a structural role in the linking the CYTOSKELETON to the EXTRACELLULAR MATRIX.

Top Publications

  1. Fort P, Sene A, Pannicke T, Roux M, Forster V, Mornet D, et al. Kir4.1 and AQP4 associate with Dp71- and utrophin-DAPs complexes in specific and defined microdomains of Müller retinal glial cell membrane. Glia. 2008;56:597-610 pubmed publisher
    ..1 in Müller cell end-feet membranes. They also show that despite its close relationship to the dystrophin proteins and its correlated upregulation, utrophin is only partially compensating for the absence of Dp71 in Müller glial cells. ..
  2. Rafii M, Hagiwara H, Mercado M, Seo N, Xu T, Dugan T, et al. Biglycan binds to alpha- and gamma-sarcoglycan and regulates their expression during development. J Cell Physiol. 2006;209:439-47 pubmed
    ..We conclude that biglycan is a ligand for two members of the sarcoglycan complex and regulates their expression at discrete developmental ages. ..
  3. Waite A, Tinsley C, Locke M, Blake D. The neurobiology of the dystrophin-associated glycoprotein complex. Ann Med. 2009;41:344-59 pubmed publisher
    ..In this review we describe the recent progress in defining the role of the DGC and associated proteins in the brain. ..
  4. Barton E. Impact of sarcoglycan complex on mechanical signal transduction in murine skeletal muscle. Am J Physiol Cell Physiol. 2006;290:C411-9 pubmed
    ..This study provides evidence that the SGs are involved in the transduction of mechanical information in skeletal muscle, potentially unique from the entire DGC...
  5. Amali A, Lin C, Chen Y, Wang W, Gong H, Rekha R, et al. Overexpression of Myostatin2 in zebrafish reduces the expression of dystrophin associated protein complex (DAPC) which leads to muscle dystrophy. J Biomed Sci. 2008;15:595-604 pubmed publisher
    ..The RT-PCR and in situ hybridization showed concurrent reduction of dystrophin associated protein complex (DAPC)...
  6. 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
    Dystrophin and Dystroglycan are the two central components of the multimeric Dystrophin Associated Protein Complex, or DAPC, that is thought to provide a mechanical link between the extracellular matrix and the actin cytoskeleton, ..
  7. Haenggi T, Fritschy J. Role of dystrophin and utrophin for assembly and function of the dystrophin glycoprotein complex in non-muscle tissue. Cell Mol Life Sci. 2006;63:1614-31 pubmed
    ..Here, we focus on recent studies of the DGC in brain, blood-brain barrier and choroid plexus, retina, and kidney and discuss the role of dystrophin isoforms and utrophin for assembly of the complex in these tissues...
  8. Sabourin J, Lamiche C, Vandebrouck A, Magaud C, Rivet J, Cognard C, et al. Regulation of TRPC1 and TRPC4 cation channels requires an alpha1-syntrophin-dependent complex in skeletal mouse myotubes. J Biol Chem. 2009;284:36248-61 pubmed publisher
    ..Cation channels with DAPC form a signaling complex that modulates cation entry and may be crucial for normal calcium homeostasis in skeletal muscles...
  9. Ito M, Ohno K. Protein-anchoring therapy to target extracellular matrix proteins to their physiological destinations. Matrix Biol. 2018;68-69:628-636 pubmed publisher
    ..We propose that protein-anchoring therapy could be applied to hereditary/acquired defects in ECM and secreted proteins, as well as therapeutic overexpression of such factors. ..

More Information


  1. Zhou Y, Oak S, Senogles S, Jarrett H. Laminin-alpha1 globular domains 3 and 4 induce heterotrimeric G protein binding to alpha-syntrophin's PDZ domain and alter intracellular Ca2+ in muscle. Am J Physiol Cell Physiol. 2005;288:C377-88 pubmed
    ..These observations suggest that DGC is a matrix laminin, G protein-coupled receptor. ..
  2. Kakarla S, Rice K, Katta A, Paturi S, Wu M, Kolli M, et al. Possible molecular mechanisms underlying age-related cardiomyocyte apoptosis in the F344XBN rat heart. J Gerontol A Biol Sci Med Sci. 2010;65:147-55 pubmed publisher
    ..Taken together, these data suggest that aging-related increases in cardiac apoptotic activity model may be due, at least in part, to age-associated changes in DGC structure. ..
  3. Fuentes Mera L, Rodríguez Muñoz R, González Ramírez R, Garcia Sierra F, Gonzalez E, Mornet D, et al. Characterization of a novel Dp71 dystrophin-associated protein complex (DAPC) present in the nucleus of HeLa cells: members of the nuclear DAPC associate with the nuclear matrix. Exp Cell Res. 2006;312:3023-35 pubmed
    ..The association of members of the nuclear DAPC with the nuclear matrix indicates that they may work as scaffolding proteins involved in nuclear architecture. ..
  4. Brown R, Grant P, Pierson C. Case records of the Massachusetts General Hospital. Case 35-2006. A newborn boy with hypotonia. N Engl J Med. 2006;355:2132-42 pubmed
  5. Batchelor C, Winder S. Sparks, signals and shock absorbers: how dystrophin loss causes muscular dystrophy. Trends Cell Biol. 2006;16:198-205 pubmed
  6. Petkova M, Morales Gonzales S, Relizani K, Gill E, Seifert F, Radke J, et al. Characterization of a Dmd (EGFP) reporter mouse as a tool to investigate dystrophin expression. Skelet Muscle. 2016;6:25 pubmed publisher
    ..The novel dystrophin reporter mouse provides a valuable tool for direct visualization of dystrophin expression and will allow the study of dystrophin expression in vivo and in vitro in various tissues by live cell imaging. ..
  7. Shah F, Berggren D, Holmlund T, Levring Jäghagen E, Stål P. Unique expression of cytoskeletal proteins in human soft palate muscles. J Anat. 2016;228:487-94 pubmed publisher
  8. Cerecedo D, Martínez Rojas D, Chávez O, Martínez Pérez F, Garcia Sierra F, Rendon A, et al. Platelet adhesion: structural and functional diversity of short dystrophin and utrophins in the formation of dystrophin-associated-protein complexes related to actin dynamics. Thromb Haemost. 2005;94:1203-12 pubmed
    ..Participation of both Dp71d/Dp71delta110m approximately DAPC and Up400/Up71 approximately DAPC in the biological roles of the platelets is discussed. ..
  9. Malerba A, Thorogood F, Dickson G, Graham I. Dosing regimen has a significant impact on the efficiency of morpholino oligomer-induced exon skipping in mdx mice. Hum Gene Ther. 2009;20:955-65 pubmed publisher
    ..These results clearly demonstrate the key role of the optimization of dosing regimen for the systemic administration of PMO in patients, and support the clinical feasibility of this approach with naked PMO...
  10. Iwata Y, Shigekawa M, Wakabayashi S. Cardiac syntrophin isoforms: species-dependent expression, association with dystrophin complex and subcellular localization. Mol Cell Biochem. 2005;268:59-66 pubmed
  11. Wang B, Li J, Qiao C, Chen C, Hu P, Zhu X, et al. A canine minidystrophin is functional and therapeutic in mdx mice. Gene Ther. 2008;15:1099-106 pubmed publisher
    ..Our results, thus, demonstrated the functionality and therapeutic potential of the canine minidystrophin and paved its way for further testing in the GRMD dog model. ..
  12. Farrell A. Wasting away. Nat Med. 2005;11:1285 pubmed
  13. Bhattacharya S, Das A, Bagchi A. In-silico structural analysis of E509K mutation in LARGE and T192M mutation in Alpha Dystroglycan in the inhibition of glycosylation of Alpha Dystroglycan by LARGE. Comput Biol Chem. 2016;64:313-321 pubmed publisher
    ..Glycoprotein ?-DG is the receptor protein in the Dystrophin Associated Protein Complex (DAPC), a macromolecular gathering on muscle cell membrane to form a bridge between extracellular ..
  14. Groh S, Zong H, Goddeeris M, Lebakken C, Venzke D, Pessin J, et al. Sarcoglycan complex: implications for metabolic defects in muscular dystrophies. J Biol Chem. 2009;284:19178-82 pubmed publisher
  15. Lovering R, Porter N, Bloch R. The muscular dystrophies: from genes to therapies. Phys Ther. 2005;85:1372-88 pubmed
    ..This perspective covers some of the basic molecular biological advances together with the clinical manifestations of the muscular dystrophies and the latest approaches to their management. ..
  16. Ramelli G. Dystrobrevin isoform expression in patients with neuromuscular disease. Clin Neuropathol. 2010;29:254-61 pubmed
    ..Immunolabeling of dystrobrevin could be a useful marker in the diagnostic of neuromuscular diseases. ..
  17. Chandrasekharan K, Martin P. Genetic defects in muscular dystrophy. Methods Enzymol. 2010;479:291-322 pubmed publisher
    ..Here we will review the mouse models available for the study of this group of diseases and outline the methodologies required to describe disease phenotypes. ..
  18. 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
    ..Nuclear envelope localization of Dp71 and DAPs might be involved in the nuclear envelope-associated functions, such as nuclear structure and modulation of nuclear processes. ..
  19. Rouillon J, Poupiot J, Zocevic A, Amor F, Léger T, García C, et al. Serum proteomic profiling reveals fragments of MYOM3 as potential biomarkers for monitoring the outcome of therapeutic interventions in muscular dystrophies. Hum Mol Genet. 2015;24:4916-32 pubmed publisher
    ..These data suggest that the MYOM3 fragments hold promise for minimally invasive assessment of experimental therapies for DMD and other neuromuscular disorders. ..
  20. Lanciotti A, Brignone M, Camerini S, Serafini B, Macchia G, Raggi C, et al. MLC1 trafficking and membrane expression in astrocytes: role of caveolin-1 and phosphorylation. Neurobiol Dis. 2010;37:581-95 pubmed publisher
    ..Because pathological mutations prevent MLC1 membrane expression, the identification of substances regulating MLC1 intracellular trafficking is potentially relevant for the therapy of MLC. ..
  21. Tidball J, Wehling Henricks M. Expression of a NOS transgene in dystrophin-deficient muscle reduces muscle membrane damage without increasing the expression of membrane-associated cytoskeletal proteins. Mol Genet Metab. 2004;82:312-20 pubmed
    ..Together, these findings indicate that the NOS transgene does not reduce dystrophinopathy by increasing the expression of compensatory, structural proteins. ..
  22. Rambukkana A. Mycobacterium leprae-induced demyelination: a model for early nerve degeneration. Curr Opin Immunol. 2004;16:511-8 pubmed
    ..The identification of the M. leprae-targeted Schwann cell receptor, dystroglycan, and its associated molecules in myelination, demyelination and axonal functions suggests a role for these molecules in early nerve degeneration. ..
  23. Ferreira L, Marie S, Liu E, Resende M, Carvalho M, Scaff M, et al. Dystrophin-glycoproteins associated in congenital muscular dystrophy: immunohistochemical analysis of 59 Brazilian cases. Arq Neuropsiquiatr. 2005;63:791-800 pubmed
  24. Lee I, Lehner B, Crombie C, Wong W, Fraser A, Marcotte E. A single gene network accurately predicts phenotypic effects of gene perturbation in Caenorhabditis elegans. Nat Genet. 2008;40:181-8 pubmed publisher
    ..We conclude that an analogous network for human genes might be similarly predictive and thus facilitate identification of disease genes and rational therapeutic targets. ..
  25. Gawor M, Proszynski T. The molecular cross talk of the dystrophin-glycoprotein complex. Ann N Y Acad Sci. 2017;: pubmed publisher
    ..Here, we focus on the role of the DGC complex in NMJ and skeletal muscle physiology and discuss the novel components that are associated with the complex...
  26. Quaranta M, Spinello I, Paolillo R, Macchia G, Boe A, Ceccarini M, et al. Identification of ?-Dystrobrevin as a Direct Target of miR-143: Involvement in Early Stages of Neural Differentiation. PLoS ONE. 2016;11:e0156325 pubmed publisher
  27. Kim H, Pierce Shimomura J, Oh H, Johnson B, Goodman M, McIntire S. The dystrophin complex controls bk channel localization and muscle activity in Caenorhabditis elegans. PLoS Genet. 2009;5:e1000780 pubmed publisher
    ..Our results indicate that the DAPC modulates muscle excitability by localizing the SLO-1 channel to calcium-rich regions of C. elegans muscle. ..
  28. Boor I, Nagtegaal M, Kamphorst W, van der Valk P, Pronk J, van Horssen J, et al. MLC1 is associated with the dystrophin-glycoprotein complex at astrocytic endfeet. Acta Neuropathol. 2007;114:403-10 pubmed
    ..We demonstrated a direct protein interaction between MLC1 and Kir4.1. From these results we conclude that MLC1 is associated with the DGC at astrocytic endfeet...
  29. Sandonà D, Betto R. Sarcoglycanopathies: molecular pathogenesis and therapeutic prospects. Expert Rev Mol Med. 2009;11:e28 pubmed publisher
    ..This review summarises the etiopathogenesis of sarcoglycanopathies and highlights the quality control machinery as a potential pharmacological target for therapy of these genetic disorders. ..
  30. Driss A, Charrier L, Yan Y, Nduati V, Sitaraman S, Merlin D. Dystroglycan receptor is involved in integrin activation in intestinal epithelia. Am J Physiol Gastrointest Liver Physiol. 2006;290:G1228-42 pubmed
    ..Collectively, these novel results indicate that dystroglycans play important roles in the regulation of interactions between intestinal epithelial cells and the extracellular matrix. ..
  31. Miller G, Wang E, Nassar K, Peter A, Crosbie R. Structural and functional analysis of the sarcoglycan-sarcospan subcomplex. Exp Cell Res. 2007;313:639-51 pubmed
    ..We propose a model whereby sarcospan forms homo-oligomers that cluster the components of the dystrophin-glycoprotein complex within the membrane. ..
  32. Annese T, Corsi P, Ruggieri S, Tamma R, Marinaccio C, Picocci S, et al. Isolation and characterization of neural stem cells from dystrophic mdx mouse. Exp Cell Res. 2016;343:190-207 pubmed publisher
  33. Pilgram G, Potikanond S, Baines R, Fradkin L, Noordermeer J. The roles of the dystrophin-associated glycoprotein complex at the synapse. Mol Neurobiol. 2010;41:1-21 pubmed publisher
    ..The challenges ahead include the integration of the behavioral and mechanistic studies and the use of this information to identify therapeutic targets...
  34. Rubi L, Koenig X, Kubista H, Todt H, Hilber K. Decreased inward rectifier potassium current IK1 in dystrophin-deficient ventricular cardiomyocytes. Channels (Austin). 2017;11:101-108 pubmed publisher
    ..This finding represents the first functional evidence for a significant role of the DAPC in the regulation of Kir2.x channels. ..
  35. Gao Y, Wineman A, Waas A. Mechanics of muscle injury induced by lengthening contraction. Ann Biomed Eng. 2008;36:1615-23 pubmed publisher
    ..Dystrophic muscles are more susceptible to contraction induced injury due to lack of DAG complex in lateral linkage. ..
  36. Estrada Mena F, Rodríguez A, Mendoza Lorenzo P, Neri Gomez T, Manjarrez Gutierrez G, Perez Ortiz A, et al. Effects of (-)-epicatechin on frontal cortex DAPC and dysbindin of the mdx mice. Neurosci Lett. 2017;658:142-149 pubmed publisher
    ..Altogether, results suggest that Epi is capable of restoring pre-frontal cortex DAPC and dysbindin levels of mdx mice towards that of healthy brains. The functional implications of such studies warrant further investigation. ..
  37. Percival J, Anderson K, Gregorevic P, Chamberlain J, Froehner S. Functional deficits in nNOSmu-deficient skeletal muscle: myopathy in nNOS knockout mice. PLoS ONE. 2008;3:e3387 pubmed publisher
    ..Our study suggests that restoration of sarcolemmal nNOSmu expression in dystrophic muscles may be more important than previously appreciated and that it should be a feature of any fully effective gene therapy-based intervention...
  38. Rice K, Preston D, Neff D, Norton M, Blough E. Age-related dystrophin-glycoprotein complex structure and function in the rat extensor digitorum longus and soleus muscle. J Gerontol A Biol Sci Med Sci. 2006;61:1119-29 pubmed
    ..Together, these data suggest that aging is associated with alterations in EDL and soleus DGC protein content and localization. These results may implicate the DGC as playing a role in age-associated skeletal muscle remodeling. ..
  39. Daicho T, Daisho Y, Kojima S, Takano S, Tejima Y, Marunouchi T, et al. Alterations in dystrophin-related glycoproteins in development of right ventricular failure in rats. J Pharmacol Sci. 2009;111:405-15 pubmed
  40. Sulyok E, Vajda Z, Doczi T, Nielsen S. Aquaporins and the central nervous system. Acta Neurochir (Wien). 2004;146:955-60 pubmed
  41. Bozzi M, Morlacchi S, Bigotti M, Sciandra F, Brancaccio A. Functional diversity of dystroglycan. Matrix Biol. 2009;28:179-87 pubmed publisher