Localization of the DMDL gene-encoded dystrophin-related protein using a panel of nineteen monoclonal antibodies: presence at neuromuscular junctions, in the sarcolemma of dystrophic skeletal muscle, in vascular and other smooth muscles, and in proliferating brain cell lines

doi:10.1083/jcb.115.6.1695

This Article

	Full Text (PDF, 1124K)
	Alert me when this article is cited
	Citation Map

Services

	Email this article
	Similar articles in this journal
	Similar articles in PubMed
	Alert me to new content in the JCB
	Download to citation manager

Citing Articles

	Citing Articles via HighWire
	Citing Articles via CrossRef
	Citing Articles via Google Scholar

Google Scholar

	Articles by Nguyen, T. M.
	Articles by Morris, G. E.
	Search for Related Content

PubMed

	PubMed Citation
	Articles by Nguyen, T. M.
	Articles by Morris, G. E.


Social Bookmarking

	What's this?

ARTICLES

Localization of the DMDL gene-encoded dystrophin-related protein using a panel of nineteen monoclonal antibodies: presence at neuromuscular junctions, in the sarcolemma of dystrophic skeletal muscle, in vascular and other smooth muscles, and in proliferating brain cell lines

TM Nguyen, JM Ellis, DR Love, KE Davies, KC Gatter, G Dickson and GE Morris
Research Division, N. E. Wales Institute, Deeside, Clwyd, Great Britain.

mAbs have been raised against different epitopes on the protein product of the DMDL gene, which is an autosomal homologue of the X-linked DMD gene for dystrophin. These antibodies provide direct evidence that DMDL protein is localized near acetylcholine receptors at neuromuscular junctions in normal and mdx mouse intercostal muscle. The primary location in tissues other than skeletal muscle is smooth muscle, especially in the vascular system, which may account for the wide tissue distribution previously demonstrated by Western blotting. The DMDL protein was undetectable in the nonjunctional sarcolemma of normal human muscle, but was observed in nonjunctional sarcolemma of Duchenne muscular dystrophy patients, where dystrophin itself is absent or greatly reduced. The expression of DMDL protein is not restricted to smooth and skeletal muscle, however, since relatively large amounts are present in transformed brain cell lines of both glial and Schwann cell origin. This contrasts with the low levels of DMDL protein in adult brain tissue.
Add to CiteULike CiteULike Add to Complore Complore Add to Connotea Connotea Add to Del.icio.us Del.icio.us Add to Digg Digg Facebook Add to Reddit Reddit Add to Technorati Technorati Twitter What's this?

This article has been cited by other articles:

Lu, Y., Tian, C., Danialou, G., Gilbert, R., Petrof, B. J., Karpati, G., Nalbantoglu, J. (2008). Targeting Artificial Transcription Factors to the Utrophin A Promoter: EFFECTS ON DYSTROPHIC PATHOLOGY AND MUSCLE FUNCTION. J. Biol. Chem. 283: 34720-34727 [Abstract] [Full Text]
Chakkalakal, J. V., Miura, P., Belanger, G., Michel, R. N., Jasmin, B. J. (2008). Modulation of utrophin A mRNA stability in fast versus slow muscles via an AU-rich element and calcineurin signaling. Nucleic Acids Res 36: 826-838 [Abstract] [Full Text]
McCarthy, J. J., Esser, K. A., Andrade, F. H. (2007). MicroRNA-206 is overexpressed in the diaphragm but not the hindlimb muscle of mdx mouse. Am. J. Physiol. Cell Physiol. 293: C451-C457 [Abstract] [Full Text]
Martinez-Martinez, P., Losen, M., Duimel, H., Frederik, P., Spaans, F., Molenaar, P., Vincent, A., De Baets, M. H. (2007). Overexpression of Rapsyn in Rat Muscle Increases Acetylcholine Receptor Levels in Chronic Experimental Autoimmune Myasthenia Gravis. Am. J. Pathol. 170: 644-657 [Abstract] [Full Text]
Driss, A., Charrier, L., Yan, Y., Nduati, V., Sitaraman, S., Merlin, D. (2006). Dystroglycan receptor is involved in integrin activation in intestinal epithelia. Am. J. Physiol. Gastrointest. Liver Physiol. 290: G1228-G1242 [Abstract] [Full Text]
Kleopa, K. A., Drousiotou, A., Mavrikiou, E., Ormiston, A., Kyriakides, T. (2006). Naturally occurring utrophin correlates with disease severity in Duchenne muscular dystrophy. Hum Mol Genet 15: 1623-1628 [Abstract] [Full Text]
Chakkalakal, J. V., Michel, S. A., Chin, E. R., Michel, R. N., Jasmin, B. J. (2006). Targeted inhibition of Ca2+/calmodulin signaling exacerbates the dystrophic phenotype in mdx mouse muscle. Hum Mol Genet 15: 1423-1435 [Abstract] [Full Text]
Chakkalakal, J. V., Thompson, J., Parks, R. J., Jasmin, B. J. (2005). Molecular, cellular, and pharmacological therapies for Duchenne/Becker muscular dystrophies. FASEB J. 19: 880-891 [Abstract] [Full Text]
De Luca, A., Nico, B., Liantonio, A., Didonna, M. P., Fraysse, B., Pierno, S., Burdi, R., Mangieri, D., Rolland, J.-F., Camerino, C., Zallone, A., Confalonieri, P., Andreetta, F., Arnoldi, E., Courdier-Fruh, I., Magyar, J. P., Frigeri, A., Pisoni, M., Svelto, M., Conte Camerino, D. (2005). A Multidisciplinary Evaluation of the Effectiveness of Cyclosporine A in Dystrophic Mdx Mice. Am. J. Pathol. 166: 477-489 [Abstract] [Full Text]
Krag, T. O. B., Bogdanovich, S., Jensen, C. J., Fischer, M. D., Hansen-Schwartz, J., Javazon, E. H., Flake, A. W., Edvinsson, L., Khurana, T. S. (2004). Heregulin ameliorates the dystrophic phenotype in mdx mice. Proc. Natl. Acad. Sci. USA 101: 13856-13860 [Abstract] [Full Text]
Ward, C. J., Yuan, D., Masyuk, T. V., Wang, X., Punyashthiti, R., Whelan, S., Bacallao, R., Torra, R., LaRusso, N. F., Torres, V. E., Harris, P. C. (2003). Cellular and subcellular localization of the ARPKD protein; fibrocystin is expressed on primary cilia. Hum Mol Genet 12: 2703-2710 [Abstract] [Full Text]
Gilbert, R., Dudley, R. W. R., Liu, A.-B., Petrof, B. J., Nalbantoglu, J., Karpati, G. (2003). Prolonged dystrophin expression and functional correction of mdx mouse muscle following gene transfer with a helper-dependent (gutted) adenovirus-encoding murine dystrophin. Hum Mol Genet 12: 1287-1299 [Abstract] [Full Text]
Guyon, J. R., Mosley, A. N., Zhou, Y., O'Brien, K. F., Sheng, X., Chiang, K., Davidson, A. J., Volinski, J. M., Zon, L. I., Kunkel, L. M. (2003). The dystrophin associated protein complex in zebrafish. Hum Mol Genet 12: 601-615 [Abstract] [Full Text]
Furling, D., Lam, L. T., Agbulut, O., Butler-Browne, G. S., Morris, G. E. (2003). Changes in Myotonic Dystrophy Protein Kinase Levels and Muscle Development in Congenital Myotonic Dystrophy. Am. J. Pathol. 162: 1001-1009 [Abstract] [Full Text]
Austin, R. C., Fox, J. E. B., Werstuck, G. H., Stafford, A. R., Bulman, D. E., Dally, G. Y., Ackerley, C. A., Weitz, J. I., Ray, P. N. (2002). Identification of Dp71 Isoforms in the Platelet Membrane Cytoskeleton. POTENTIAL ROLE IN THROMBIN-MEDIATED PLATELET ADHESION. J. Biol. Chem. 277: 47106-47113 [Abstract] [Full Text]
Weir, A. P., Burton, E. A., Harrod, G., Davies, K. E. (2002). A- and B-utrophin Have Different Expression Patterns and Are Differentially Up-regulated in mdx Muscle. J. Biol. Chem. 277: 45285-45290 [Abstract] [Full Text]
CROSBIE, R. H., DOVICO, S. A., FLANAGAN, J. D., CHAMBERLAIN, J. S., OWNBY, C. L., CAMPBELL, K. P. (2002). Characterization of aquaporin-4 in muscle and muscular dystrophy. FASEB J. 16: 943-949 [Abstract] [Full Text]
Newby, L. J., Streets, A. J., Zhao, Y., Harris, P. C., Ward, C. J., Ong, A. C. M. (2002). Identification, Characterization, and Localization of a Novel Kidney Polycystin-1-Polycystin-2 Complex. J. Biol. Chem. 277: 20763-20773 [Abstract] [Full Text]
Rybakova, I. N., Patel, J. R., Davies, K. E., Yurchenco, P. D., Ervasti, J. M. (2002). Utrophin Binds Laterally along Actin Filaments and Can Couple Costameric Actin with Sarcolemma When Overexpressed in Dystrophin-deficient Muscle. Mol. Biol. Cell 13: 1512-1521 [Abstract] [Full Text]
Blake, D. J., Weir, A., Newey, S. E., Davies, K. E. (2002). Function and Genetics of Dystrophin and Dystrophin-Related Proteins in Muscle. Physiol. Rev. 82: 291-329 [Abstract] [Full Text]
Moiseeva, E. P. (2001). Adhesion receptors of vascular smooth muscle cells and their functions. Cardiovasc Res 52: 372-386 [Abstract] [Full Text]
Gramolini, A. O., Belanger, G., Thompson, J. M., Chakkalakal, J. V., Jasmin, B. J. (2001). Increased expression of utrophin in a slow vs. a fast muscle involves posttranscriptional events. Am. J. Physiol. Cell Physiol. 281: C1300-C1309 [Abstract] [Full Text]
Young, P. J., Man, N. t., Lorson, C. L., Le, T. T., Androphy, E. J., Burghes, A. H.M., Morris, G. E. (2000). The exon 2b region of the spinal muscular atrophy protein, SMN, is involved in self-association and SIP1 binding. Hum Mol Genet 9: 2869-2877 [Abstract] [Full Text]
EBIHARA, S., GUIBINGA, G.-H., GILBERT, R., NALBANTOGLU, J., MASSIE, B., KARPATI, G., PETROF, B. J. (2000). Differential effects of dystrophin and utrophin gene transfer in immunocompetent muscular dystrophy (mdx) mice. Physiol. Genomics 3: 133-144 [Abstract] [Full Text]
Lam, L.T., Pham, Y.C.N., Man, N. t., Morris, G.E. (2000). Characterization of a monoclonal antibody panel shows that the myotonic dystrophy protein kinase, DMPK, is expressed almost exclusively in muscle and heart. Hum Mol Genet 9: 2167-2173 [Abstract] [Full Text]
Raats, C. J. I., van den Born, J., Bakker, M. A. H., Oppers-Walgreen, B., Pisa, B. J. M., Dijkman, H. B. P. M., Assmann, K. J. M., Berden, J. H. M. (2000). Expression of Agrin, Dystroglycan, and Utrophin in Normal Renal Tissue and in Experimental Glomerulopathies. Am. J. Pathol. 156: 1749-1765 [Abstract] [Full Text]
Khurana, T. S., Rosmarin, A. G., Shang, J., Krag, T. O.�B., Das, S., Gammeltoft, S. (1999). Activation of Utrophin Promoter by Heregulin via the ets-related Transcription Factor Complex GA-binding Protein alpha /beta. Mol. Biol. Cell 10: 2075-2086 [Abstract] [Full Text]
Kachinsky, A. M., Froehner, S. C., Milgram, S. L. (1999). A PDZ-containing Scaffold Related to the Dystrophin Complex at the Basolateral Membrane of Epithelial Cells. JCB 145: 391-402 [Abstract] [Full Text]
Crosbie, R. H., Lebakken, C. S., Holt, K. H., Venzke, D. P., Straub, V., Lee, J. C., Grady, R. M., Chamberlain, J. S., Sanes, J. R., Campbell, K. P. (1999). Membrane Targeting and Stabilization of Sarcospan Is Mediated by the Sarcoglycan Subcomplex. JCB 145: 153-165 [Abstract] [Full Text]
Williams, M. W., Bloch, R. J. (1999). Extensive but Coordinated Reorganization of the Membrane Skeleton in Myofibers of Dystrophic (mdx) Mice. JCB 144: 1259-1270 [Abstract] [Full Text]
Gramolini, A. O., Angus, L. M., Schaeffer, L., Burton, E. A., Tinsley, J. M., Davies, K. E., Changeux, J.-P., Jasmin, B. J. (1999). Induction of utrophin gene expression by heregulin in skeletal muscle cells: Role of the N-box motif and GA binding protein. Proc. Natl. Acad. Sci. USA 96: 3223-3227 [Abstract] [Full Text]
Peters, M. F., Sadoulet-Puccio, H. M., Mark Grady, R., Kramarcy, N. R., Kunkel, L. M., Sanes, J. R., Sealock, R., Froehner, S. C. (1998). Differential Membrane Localization and Intermolecular Associations of {alpha}-Dystrobrevin Isoforms in Skeletal Muscle. JCB 142: 1269-1278 [Abstract] [Full Text]
Gramolini, A. O., Burton, E. A., Tinsley, J. M., Ferns, M. J., Cartaud, A., Cartaud, J., Davies, K. E., Lunde, J. A., Jasmin, B. J. (1998). Muscle and Neural Isoforms of Agrin Increase Utrophin Expression in Cultured Myotubes via a Transcriptional Regulatory Mechanism. J. Biol. Chem. 273: 736-743 [Abstract] [Full Text]
Nawrotzki, R, Loh, N., Ruegg, M., Davies, K., Blake, D. (1998). Characterisation of alpha-dystrobrevin in muscle. J. Cell Sci. 111: 2595-2605 [Abstract]
Blake, D. J., Nawrotzki, R., Loh, N. Y., Gorecki, D. C., Davies, K. E. (1998). beta -dystrobrevin, a member of the dystrophin-related protein�family. Proc. Natl. Acad. Sci. USA 95: 241-246 [Abstract] [Full Text]
Cohen, M. W., Jacobson, C., Yurchenco, P. D., Morris, G. E., Carbonetto, S. (1997). Laminin-induced Clustering of Dystroglycan on Embryonic Muscle Cells: Comparison with Agrin-induced Clustering. JCB 136: 1047-1058 [Abstract] [Full Text]
Grady, R. M., Merlie, J. P., Sanes, J. R. (1997). Subtle Neuromuscular Defects in Utrophin-deficient Mice. JCB 136: 871-882 [Abstract] [Full Text]
Deconinck, A. E., Potter, A. C., Tinsley, J. M., Wood, S. J., Vater, R., Young, C., Metzinger, L., Vincent, A., Slater, C. R., Davies, K. E. (1997). Postsynaptic Abnormalities at the Neuromuscular Junctions of Utrophin-deficient Mice. JCB 136: 883-894 [Abstract] [Full Text]
Meng, H., Leddy, J. J., Frank, J., Holland, P., Tuana, B. S. (1996). The Association of Cardiac Dystrophin with Myofibrils/Z-disc Regions in Cardiac Muscle Suggests a Novel Role in the Contractile Apparatus. J. Biol. Chem. 271: 12364-12371 [Abstract] [Full Text]
Blake, D. J., Nawrotzki, R., Peters, M. F., Froehner, S. C., Davies, K. E. (1996). Isoform Diversity of Dystrobrevin, the Murine 87-kDa Postsynaptic Protein. J. Biol. Chem. 271: 7802-7810 [Abstract] [Full Text]
Hopf, C., Hoch, W. (1996). Agrin Binding to alpha-Dystroglycan. J. Biol. Chem. 271: 5231-5236 [Abstract] [Full Text]
Earnest, J. P., Santos, G. F., Zuerbig, S., Fox, J. E. B. (1995). Dystrophin-related Protein in the Platelet Membrane Skeleton. J. Biol. Chem. 270: 27259-27265 [Abstract] [Full Text]
Belkin, A. M., Burridge, K. (1995). Association of Aciculin with Dystrophin and Utrophin. J. Biol. Chem. 270: 6328-6337 [Abstract] [Full Text]
Winder, S., Hemmings, L, Maciver, S., Bolton, S., Tinsley, J., Davies, K., Critchley, D., Kendrick-Jones, J (1995). Utrophin actin binding domain: analysis of actin binding and cellular targeting. J. Cell Sci. 108: 63-71 [Abstract]
Khurana, T., Kunkel, L., Frederickson, A., Carbonetto, S, Watkins, S. (1995). Interaction of chromosome-6-encoded dystrophin related protein with the extracellular matrix. J. Cell Sci. 108: 173-185 [Abstract]
Law, D., Allen, D., Tidball, J. (1994). Talin, vinculin and DRP (utrophin) concentrations are increased at mdx myotendinous junctions following onset of necrosis. J. Cell Sci. 107: 1477-1483 [Abstract]
Dickson, G., Azad, A., Morris, G. E., Simon, H., Noursadeghi, M., Walsh, F. S. (1992). Co-localization and molecular association of dystrophin with laminin at the surface of mouse and human myotubes. J. Cell Sci. 103: 1223-1233 [Abstract]
Luna, E., Hitt, A. (1992). Cytoskeleton--plasma membrane interactions. Science 258: 955-964 [Abstract]
Peng, H. B., Chen, Q. (1992). Induction of dystrophin localization in cultured Xenopus muscle cells by latex beads. J. Cell Sci. 103: 551-563 [Abstract]
McDearmon, E. L., Combs, A. C., Ervasti, J. M. (2001). Differential Vicia villosa Agglutinin Reactivity Identifies Three Distinct Dystroglycan Complexes in Skeletal Muscle. J. Biol. Chem. 276: 35078-35086 [Abstract] [Full Text]