The membrane-cytoskeleton interface: The role of dystrophin and utrophin

Journal of Muscle Research and Cell Motility

Volumn 18, Issue 6, 1997, Pages 617-629

  Winder, Steven J ^a  

^a UNIVERSITY OF EDINBURGH   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

DYSTROPHIN; UTROPHIN;

ANIMAL MODEL; ANIMAL TISSUE; CONTROLLED STUDY; HISTOCHEMISTRY; MOUSE; MUSCLE; MUSCULAR DYSTROPHY; MUTATION; NEUROMUSCULAR SYNAPSE; NONHUMAN; PHENOTYPE; PRIORITY JOURNAL; REVIEW; TRANSGENIC ANIMAL;

EID: 0030668762     PISSN: 01424319     EISSN: None     Source Type: Journal    
DOI: 10.1023/A:1018627705273     Document Type: Review

References (108)

1. AHN, A. H. & KUNKEL, L. M. (1995) Syntrophin binds to an alternatively spliced exon of dystrophin. J. Cell Biol. 128, 363-71.
2. AMALFITANO, A., RAFAEL, J. A. & CHAMBERLAIN. J. S. (1997) Structure and mutation of the dystrophin gene. In Dystrophin: Gene, Protein and Cell Biology (edited by LUCY, J. A. & BROWN, S. C.), pp. 1-26. Cambridge: Cambridge University Press.
3. ANDRE, B. & SPRINGAEL, J. Y. (1994) WWP, a new amino acid motif present in single or multiple copies in various proteins including dystrophin and the SH3-binding Yes-associated protein YAP65. Biochem. Biophys. Res. Commun. 205, 1201-5.
4. ASCADI, G., DICKSON, G., LOVE, D. R., JANI, A., WALSH, F. S., GURUSINGHE, A., WOLFF, J. A. & DAVIES. K. F. (1991) Human dystrophin expression in mdx mice after intramuscular injection of DNA constructs. Nature 352, 815-8.
5. BAR, S., BARNEA, E., LAVY, Z., NEUMAN, S., YAFFE, D. & NUDEL, U. (1990) A novel protein product of the Duchenne muscular dystrophy gene which greatly differs from known isoforms in its structure and tissue distribution. Biochem. J. 272, 557-60.
6. BEGGS, A. H., HOFFMAN, E. P., SNYDER, J. R., ARAHATA, K., SPECHT, L., SHAPIRO, F., ANGELINI, C., SUGITA, H. & KUNKEL, L. M. (1991) Exploring the molecular basis for variability among patients with Becker muscular dystrophy - dystrophin gene and protein studies. Am. J. Human Gen. 49, 54-67.
7. BLAKE, D. L., TINSLEY, L. M., DAVIES, K. E., KNIGHT, A. E., WINDER, S. J. & KENDRICK-JONES. J. (1995a) Coiledcoil regions in the carboxy terminal domains of dystrophin and related proteins: potentials for protein-protein interactions. TIBS 20, 133-5.
8. BLAKE, D. J., SCHOLFIELD. J. N., ZUELLIG, R. A., GORECKI, D. G., PHELPS, S. R., BARNARD, E. A., EDWARDS, Y. H. & DAVIES, K. E. (1995b) G-utrophin, the autosomal homologue of dystrophin DP116, is expressed in sensory ganglia and brain. Proc. Nalt Acad. Sci. USA 92, 3697-701.
9. BLAKE, D. J., TINSLEY, J. M. & DAVIES, K. E. (1996a) Utrophin: a structural and functional comparison to dystrophin. Brain Path. 6, 37-47.
10. BLAKE, D. J., NAWROTZKl, R., PETERS, M. F., FROEHNER, S. C. & DAVIES, K. E (1996b) Isoform diversity of dystrobrevin, the murine 87-kDa postsynaptic protein. J. Biol. Chem. 271, 7802-10.
11. BONNEMANN, C. G., et al. (1995) β-sarcoglycan (A3b) mutations cause autosomal recessive muscular dystrophy with loss of the sarcoglycan complex. Nature Genet. 11, 266-73.
12. BORK, P. & SUDOL, M (1994) The WW domain: a signalling site in dystrophin? Trends Biochem. Sci. 19, 531-3.
13. BYERS, T. J., LIDOV, H. G. W. & KUNKEL, L. M. (1993) An alternative dystrophin transcript specific to peripheral nerve. Nature Genet. 4, 77-81.
14. CAMPBELL, K. P. (1995) Three muscular dystrophies: loss of cytoskeleton-extracellular matrix linkage. Cell 80, 675-9.
15. CAMPBELL, K. P. & KAHL, S. D. (1989) Association of dystrophin and an integral membrane glycoprotein. Nature 338, 259-62.
16. CHEN, H. L. SUDOL, M. (1995) The WW domain of the Yes-associated protein binds a proline-rich ligand that differs from the consensus established for Src homology 3-binding modules. Proc. Natl Acad. Sci. USA 92, 7819-23.
17. COLLlNS, A. F., PEARSON, H. A, GIARDINA, P., MCDONAGH, K. T., BRUSILOW, S. W. & DOVER, G. J. (1995) Oral sodium phenylbutyrate therapy in homozygous β-thalassemia: a clinical trial. Blood 85, 43-9.
18. CORRADO, K., RAFAEL, J. A., MILLS, P. L., COLE, N. M., FAULKNER, J. A., WANG, K. & CHAMBERLAIN, J. S. (1996) Transgenic mdx mice expressing dystrophin with a deletion in the actin-binding domain display a "mild Becker" phenotype. J. Cell Biol. 134, 873-84.
19. COX, G. A., PHELPS, S. F., CHAPMAN, V. M. & CHAMBERLAIN, J. S. (1993) New mdx mutation disrupts expression of muscle and nonmuscle isoforms of dystrophin. Nature Genet. 4, 87-93.
20. COX, G. A., SUNADA, Y., CAMPBELL, K. P. & CHAMBERLAIN, J. S. (1994) Dp71 can restore the dystrophin-associated glycoprotein complex in muscle but fails to prevent dystrophy. Nature Genet. 8, 333-9.
21. DIVISON, M. D. & CRITCHLEY, D. R. (1988) Alpha-actinins and the DMD protein contain spectrin-like repeats. Cell 52, 159-60.
22. DAWSON, R. M. C., ELLIOTT, D.C., ELLIOTT, W.H. & JONES, K.M., (1986) Data for Biochemical Research. Oxford: Clarendon Press.
23. 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. (1997a) Postsynaptic abnormalities at the neuromuscular junctions of utrophin-deficient mice. J. Cell J Biol. 136, 883-940.
24. DECONINCK, A. E., RAFAEL, J. A., SKINNER, J A., BROWN, S. C., POTTER, A. C., METZINGER, L., WATT, D. L., DICKSON, J. G., TINSLEY, J. M. & DAVIES, K. E. (1997b) Utrophin-dystrophin-deficient mice as a model for Duchenne muscular dystrophy. Cell 90, 717-27.
25. DOVER, G. J., BRUSILOW, S. & CHARACHE, S. (1994) Induction of fetal hemoglobin production in subjects with sickle-cell-anemia by oral sodium phenylbutyrate. Blood 84, 339-43.
26. D'SOUZA, V. N., NGUYEN THI MAN, MORRIS, G. E., KARGES, W., PILLERS, D. A. & RAY, P. N. (1995) A novel dystrophin isoform is required for normal retinal electrophysiology. Hum. Mol. Genet. 4, 837-42.
27. EMERY, A.E.H., (1993) Duchennu Muscular Dystrophy. Oxford: Oxford University Press.
28. ENGLAND, S. B., NICHOLSON, L. V. B., JOHNSON, M. A., FORREST, S. M., LOVE., D. R., ZUBRZYCKA-GAARN, E. E., BULMAN, D. E., HARRIS, J. B. & DAVIES, K, E. (1990) Very mild muscular dystrophy associated with the deletion of 46% of dystrophin. Nature 343, 180-82.
29. ERVASTI, J. M. & CAMPBELL, K, P. (1993) A role for the dystrophin glycoprotein complex as a transmembrane linker between laminin and actin. J. Cell Biol. 122, 809-23.
30. ERVASTI, J. M., OHLENDIECK, K., KAHL, S. D., GAVER, M. G. & CAMPBELL, K. P. (1990) Deficiency of a glycoprotein component of the dystrophin complex in dystrophic muscle. Nature 345, 315-9.
31. ERVASTI, J. M , KAHL., S. D. & CAMPBELL, K., P. (1991) Purification of dystrophin from skeletal muscle. J. Biol. Chem. 266, 9161-5.
32. FABBRIZIO, E., BONET-KERRACHE, A., LEGER, J. J. & MORNET, D. (1993) Actin-dystrophin interface. Biochem. 32, 10457-63.
33. FABBRIZIO, E., PONS, F., ROBERT, A., HUGNON, G., BONET-KERRACHE, A. & MORNET, D. (1994) The dystrophin superfamily: variability and complexity. J. Muscle Res. Cell Motil. 15, 595-606.
34. GORECKI, D. C. & BARNARD, E. A. (1997) Expression of the dystrophin complex in brain. In Dystrophin: Gene, Protein and Cell Biology (edited by LUCY, J. A. & BKOVVN, S. C.), pp. 105-38. Cambridge: Cambridge University Press.
35. GORECKI, D. C., MONACO, A. P., DERRY, J. M. J., WALKER, A. P., BARNARD, E, A. & BARNARD, P. J. (1992) Expression of four alternative transcripts in human brain regions regulated by different promoters. Hum. Mol. Genet. 1, 505-10.
36. GRADY, R. M., MERLIE, J. P. & SANES, J. R. (1997a) Subtle neuromuscular defects in utrophin-deficient mice. J. Cell Biol. 136, 871-82.
37. GRADY, R. M., TENG, H., NICHOLL, M. C., CUNNINGHAM, J. C., WILKINSON, R. S. & SANES, J. R. (1997b) Skeletal and cardiac myopathies in mice lacking utrophin and dystrophin: a model for Duchenne muscular dystrophy. Cell 90, 729-38.
38. GRAMOLINI, A. O., DENNIS, C. L., TINSLEY, J. M., ROBERTSON, G. S., CARTAUD, J., DAVIES, K. E. & JASMIN, B. J. (1997) Local transcriptional control of utrophin expression at the neuromuscular synapase. J. Biol. Chem. Nature Genet. 272, 8117-20.
39. GREENBERG, D. S., SUNADA, Y., CAMPBELL, K. P., YAFFE, D. & NUDEL, U. (1994) Exogenous Dp71 restores the levels of dystrophin-associated proteins but does not alleviate muscle damage in Mdx mice. Nature Genet. 8, 340-44.
40. HAMMONDS, R. G. (1987) Protein sequence of DMD gene is related to actin binding domain of alpha-actinin. Cell 51, 1.
41. HEMMINGS, L., KUHLMAN, P. A. & CRITCHLKY, D. R. (1992) Analysis of the actin-binding domain of α-actinin by mutatgenesis and demonstration that dystrophin contains a functionally homologous domain. J. Cell Biol. 116, 1369-80.
42. HOFFMAN, E. P., BROWN, R. H. & KUNKEL, L. M. (1987) Dystrophin - the protein product of the Duchenne muscular-dystrophy locus. Cell 51, 919-28.
43. HORI, S., OHTANI, S., NGUYEN THI MAN & MORRIS, G. E. (1995) The N-terminal half of dystrophin is protected from proteolysis in situ. Biochem. Biophys. Res. Commun. 209, 1062-7.
44. IBRAGHIMOV-BESKROVNAYA, O., ERVASTI, J. M., LEVEILLE, C. J., SLAUGHTER, C. A., SERNETT, S. W. & CAMPBELL, K. P. (1992) Primary structure of dystrophin-associated glycoproteins linking dystrophin to the extracellular matrix. Nature 355, 696-702.
45. JUNG, D., YANG, B., MEYER, J., CHAMBERLAIN, J. S. & CAMPBELL, K. P. (1995) Identification and characterization on the dystrophin anchoring site on beta-dystroglycan. J. Biol. Chem. 270, 27305-10.
46. JUNG, D., DUCLOS, F., APOSTOL, B., STRAUB, V., LEE, J. C., ALLAMAND, V., VENZKE, D. P., SUNADA, Y., MOOMAW, C. R., LEVEILLE, C. J., SLAUGHTER, C. A., CRAWFORD, T. O., MCPHERSON, J. D. & CAMPBELL, K. P. (1996) Characterization of δ-sarcoglycan, a novel component of the oligomeric sarcoglycan complex involved in limb-girdle muscular dystrophy. J. Biol. Chem. 271, 32321-9.
47. KAHANA, E., FLOOD, G. & GRATZER, W. B. (1997) Physical properties of dvstrophin rod domain. Cell Motil. Cytoskel. 36, 246-52.
48. KHURANA, T. S., HOFFMAN, E. P. & KUNKEL, L. M. (1990) Identification of a chromosome-6-encoded dystrophin-related protein. J. Biol. Chem. 265, 16717-20.
49. KLAMUT, H. J., GANGOPADHYAY, S. B., WORTON, R. G. & RAY, P. N (1990) Molecular and functional analysis of the muscle-specific promoter region of the Duchenne muscular dystrophy gene. Mol. Cell. Biol. 10, 193-205.
50. KOENIG, M., HOFFMAN, E. P., BERTELSON, C. J., MONACO, A. P., FEENER, C. & KUNKEL, L. M. (1987) Complete cloning of the Duchenne muscular-dystrophy (DMD) cDNA and preliminary genomic organization of the DMD gene in normal and affected individuals. Cell 50, 509-17.
51. KOENIG, M., MONACO, A. P. & KUNKEL. L. M. (1988) The complete sequence of dystrophin predicts a rod-shaped cytoskeletal protein. Cell 53, 219-28.
52. KOENIG. M. et al. (1989) The molecular basis Duchenne versus Becker muscular-dystrophy - correlation of severity with type of deletion. Am. J. Hum. Genet. 45, 498-506.
53. r 58,000 dystrophin-associated protein (syntrophin). J. Biol. Chem. 269, 2870-6.
54. LEBART, M.-C., CASANOVA, D. & BENYAMIN, Y. (1995) Actin interaction with purified dystrophin from electric organ of Torpedo marmorata: possible resemblence with filamin-actin interface. J. Muscle Res. Cell Motil. 16, 543-52.
55. LENK, U., OXELE, K., VOIT, T., ANCKER, U., HELLNER, K.-A., SPEER, A. & HUBNER, C. (1996) A cysteine 3340 substitution in the dystroglycan-binding of dystrophin associated with Duchenne muscular dystrophy, mental retardation and absence of the ERG b-wave. Hum. Mol. Genet. 5, 973-5.
56. LIDOV, H. G. W., SELIG, S. & KUNKEL. L. M. (1995) Dp140: a novel 140 kDa ens transcript from the dystrophin locus. Hum. Mol. Genet. 4, 329-35.
57. LIM, L. E., DUCLOS, F., BROUX, O., BOURG, N., SUNADA, Y., ALLAMAND, V., MEYFR, J., RICHARD, L, MOOMAW, C., SLAUGHTER, C., TOME, F. M. S., FARDEAU, M., JACKSON, C. E., BECKMANN, J. S. & CAMPBELL, K. P. (1995) β-sarcoglycan: characterization and role in limb-girdle muscular dystrophy linked to 4q12. Nature Genet. 11, 257-65.
58. LOVE, D. R., HILL, D, F., DICKSON, G., SPURR, N. K., BYTH, B. C., MARSDEN, R. F., WALSH, F. S., EDWARDS, Y. H. & DAVIES, K. E. (1989) An autosomal transcript in skeletal muscle with homology to dystrophin. Nature 339, 55-8.
59. LOVE, D.R., MORRIS, G.E., ELLIS, J.M., FAIRBROTHER, O., MARSDEN, R.F., BLOOMFIELD, J.F., EDWARDS, Y.H., SLATER, C.P., PARRY, D.J, & DAVIES, K,E. (1991) Tissue distribution of the dystrophin-related gene product and expression in the mdx and dy mouse. Proc. Natl Acad. Sci. USA 88, 3243-7.
60. MACIAS, M. J., HYVONEN, M., BARALDI, E., SCHULTZ. J., SUDOL, M., SARASTE, M. & OSCHKINAT, H. (1996) Structure of the WW domain of a kinase-associated protein complexed with a proline-rich peptide. Nature 382, 646-9.
61. MICHALAK, M., FU, S. Y., MILNER, R, E., BUSAAN, J. I. & HANCE, J. E. (1996) Phosphorylation of the carboxyterminal region of dystrophin. Biochem. Cell Biol. 74, 431-7.
62. MILNER, R, E., BUSSAN, J. L. & MICHALAK, M. (1992) Isolation and characterisation of different C-terminal fragments of dystrophin expressed in Escherichia coli. Biochem. J. 288, 1037-44.
63. MIZUNO, Y., YOSHIDA, M., YAMAMOTO, H., HIRAI, S. & OZAWA, E. (1993) Distribution of dystrophin isoforms and dystrophin-associated proteins 43DAG (A3a) and 50DAG (A2) in various monkey tissues. J. Biochem. 114, 936-41.
64. MONACO, A. P., BERTELSON, C. J, LIECHTI-GALLATI, S., MOSER, H. & KUNKEL, I., (1988) An explanation for the phenotypic differences between patients bearing partial deletions of the DMD locus. Genomics 2, 90-95.
65. NGUYEN THI MAN, ELLIS, J. M., LOVE, D. R., DAVIES, K. E., GATTER, K. C., DICKSON, G. & MORRIS, G. E. (1991) Localization of the DMDL gene-encoded dystrophin-related protein using a panel of nineteen monoclocal antibodies: presence at neuromuscular junctions, in the sarcolemma of dystrophic skeletal muscle in vascular and other smooth muscles, and in proliferating brain cell lines. J. Cell Biol. 115, 1695-1700.
66. NGUYEN THI MAN, HELLIWELL, T. R., SIMMONS, C., WINDER, S. J., KENDRICK-JONES, J., DAVIES, K. E. & MORRIS, G. (1995) Full-length and short forms of utrophin, the dystrophin-related protein. FEBS Lett. 258, 262-6.
67. NUDEL, U., ZUK, D., EINAT, P., ZEELON, E., LEVY, Z., NEUMAN, S. & YAFFE, D. (1989) Duchenne muscular dystrophy gene product is not identical in muscle and brain. Nature 337, 76-8.
68. OHLENDIECK, K., ERVASTI, J. M., MATSUMURA, K., KAHI., S. D, LEVEILLE, C. J. & CAMPBELL, K. P. (1991) Dystrophin-related protein is localized to neuromuscular-junctions of adult skeletal-muscle. Neuron 7, 499-508.
69. PASTERNACK, C., WONG, S. & ELSON, E. L. (1995) Mechanical function of dystrophin in muscle cells. J. Cell Biol. 128, 355-61.
70. PHELPS, S. F., HAUSER, M. A., COLE, N. M., RAFAEL, J. A., HINKLE, R. T. & FAULKNER, J. A. (1995) Expression of full-length and truncated dystrophin mini-genes in transgenic mdx mice. Hum. Mol. Genet. 4, 1251-8.
71. PONTING, C. P., BLAKE, D. J., DAVIES, K. E., KENDRICKJONES, J. & WINDER, S.J. (1996) ZZ and TAZ: new-putative zinc fingers in dystrophin, CBP, p300 and other proteins. Trends Biochem. Sci. 21, 11-13.
72. PRIOR, T. W., PAPP, A. C., SNYDER, P. J., BURGHES, A. H. M., BARTOLO, C., SEDRA, M. S., WESTERN, L. M. & MENDELL, J. R. (1993) A missense mutation in the dystrophin gene in a Duchenne muscular dystrophy patient. Nature Genet. 4, 357-60.
73. RAFAEL, J. A., COX, G. A., CORRADO, K., JUNG, D., CAMP-BELL, K P. & CHAMBERLAIN, J. S. (1996) Forced expression of dystrophin deletion constructs reveals structure-function correlations. J. Cell Biol. 134, 93-102.
74. ROBERTS, R. G., GARDNER, R. J. & BOBROW, M. (1994) Searching for the 1 in 2,400,000: a review of dystrophin gene point mutations. Hum. Mutat. 4, 1-11.
75. ROBERTS, R. G., FREEMAN, T. C., KENDALL, E., VETRIE, D. L. P., DIXON, A. K., SHAW-SMITH, C., BONE, Q. & BOBROW, M. (1996) Characterization of DRP2, a novel human dystrophin homologue. Nature Genet. 13, 223-6.
76. ROSA, G., CECCARINI, M., CAVALDESI, M., ZINI, M & PETRUCCI, T. C. (1996) Localisation of the dystrophin binding site at the carboxyl terminus of β-dystroglycan. Biochem. Biophys. Res. Commun. 223, 272-7.
77. RYBAKOVA, L. N., AMMAN, K. J. & ERVASTI, J. M. (1996) A new model for the interaction of dystrophin with F-actin. J. Cell Biol. 135, 661-72.
78. SADOULET-PUCCIO, H. M. & KUNKEL, L. M. (1996) Dystrophin and its isoforms. Brain Path. 6, 25-35.
79. SARASTE. M. & MUSACCHIO, A (1994) Backwards and forwards binding. Nature Struct. Biol. 1, 835-7.
80. SENTER, L., LUISE, M., PRESOTTO, C., BETTO, R., TERESI, A., CEOLDO, S. & SALVIATI, G. (1993) Interaction of dystrophin with cytoskeletal proteins: binding to talin and actin. Biochem. Biophys. Res. Commun. 192, 899-904.
81. SONG, K. S., SCHERER, P. E., TANG, Z., OKAMOTO, T., LI, S., CHAFEL, M., CHU, C., KOHTZ, D. S. & LISSANTI, M. P. (1996) 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. 271, 15160-65.
82. SUZUKI, A., YOSHIDA, M., YAMAMOTO, H. & OZAWA, E (1992) Glycoprotein-binding site of dystrophin is confined to the cysteine-rich domain and the first half of the carboxy-terminal domain. FEBS Lett. 308, 154-60.
83. SUZUKI, A., YOSHIDA, M., HAYASHI, K., MIZUNO, Y., HAGIWARA, Y. & OZAWA, E. (1994) Molecular organisation at the glycoprotein-complex-binding site of dystrophin: three dystrophin-associated proteins bind directly to the carboxy-terminal portion of dystrophin. Eur. J. Biochem. 220, 283-92.
84. SUZUKI, A., YOSHIDA, M. & OZAWA, E,. (1995) Mammalian α1- and β1-syntrophin bind to the alternative splice prone region of the dystrophin COOH-terminus. J. Cell Biol. 128, 373-81.
85. THOMPSON, J. D., HIGGINS, D. G. & GIBSON, T. J. (1994) Clustal W: improving the sensitivity of progressive multiple sequence alignment through sequence weighting, position-specific gap penalties and weight matrix choice. Nucleic Acids Res. 22, 4673-80.
86. TINSLEY, J. M., BLAKE, D. J., ROCHE, A., FAIRBROTHER, U., RISS, J., BYTH, B. C., KNIGHT, A. E., KENDRICK-JONES, J., SUTHERS, G. K., LOVE, D. R., EDWARDS, Y. H. & DAVIES, K. E. (1992) Primary structure of dystrophin-related protein. Nature 360, 591-3.
87. TINSLEY, J. M., BLAKE, D. J. & DAVIES, K. E. (1993) Apodystrophin 3: a 2.2 kb transcript from the DMD locus encoding the dystrophin glycoprotein binding site. Hum. Mol. Genet. 2, 521-4.
88. TINSLEY, J. M., BLAKE, D. J., ZUELLIG, R. A. & DAVIES, K. E (1994) Increasing complexity of the dystrophin-associated protein complex, Proc. Natl Acad. Sci. USA 91, 8307-13.
89. TINSLEY, J. M., POTTER, A. C., PHELPS, S. R., FISHER, R., TRICKETT, J. I. & DAVIES, K. E. (1996) Amelioration of the dystrophic phenotype of max mice using a truncated utrophin transgene. Nature 384, 349-53.
90. TINSLEY, J. M., BLAKE, D. J. & DAVIES, K. E. (1997) Dystrophin, utrophin and their associated proteins. In Dystrophin: Gene, Protein and Cell Biology (edited by LUCY, J. A. & BROWN, S. C.), pp. 56-78. Cambridge: Cambridge University Press.
91. TUFTY, R. M. & KRETSINGER, R. H. (1975) Troponin and parvalbumin calcium binding regions predicted in myosin light chain and T4 iysozyme. Science 187, 167-9.
92. WAGNER, K. R., COHEN, J. B. & HUGANIR, R. L. (1993) The 87K postsynaptic membrane protein from Torpedo is a protein-tyrosine kinase substrate homologous to dystrophin. Neuron 10, 511-22.
93. WAGNER, K. R., COHEN, J. B. & HUGANIR, R. L. (1993) The 87K postsynaptic membrane protein from Torpedo is a protein-tyrosine kinase substrate homologous to dystrophin. Neuron 10, 511-22.
94. WAGNER, K. R. & HUGANIR, R. L. (1994) Tyrosine and serine phosphorylation of dystrophin and the 58 kDa protein in the postsynaptic membrane of Torpedo electric organ. J. Neurochem. 62, 1947-52.
95. WATKINS, S. C., SWARTZ, D. R. & BYERS, T. J., (1997) Localisation of dystrophin in skeletal, cardiac and smooth muscle. In Dystrophin: Gene, Protein and Cell Biology (edited by LUCY, J. A. & BROWN, S. C.), pp. 79-104. Cambridge: Cambridge University Press.
96. WAY, M., POPE, B., CROSS, R. A., KENDRICK-JONES, J. & WEEDS, A. G. (1992) Expression of the N-terminal domain of dystrophin in E. coli and demonstration of binding to F-actin. FEBS Lett. 301, 243-5.
97. WELLS, D. J., WELLS, K. E., ASANTE, E. A., TURNER, G., SUNADA, Y., CAMPBELL, K. P., WALSH, F. S. & DICKSON, G. (1995) Expression of human full-length and minidystrophin in transgenic mdx mice: implications for gene therapy of Duchenne muscular dystrophy. Hum. Mol. Genet. 4, 1245-50.
98. WINDER, S. J. (1996) Structure-function relationships in dystrophin and utrophin. Biochem. Soc. Trans. 24, 497-501.
99. WINDER, S. J. & KENDRICK-JONES, J. (1995) Calcium/ calmodulin-dependent regulation of the NH2-terminal F-actin binding domain of utrophin. FEBS Lett. 357, 125-8.
100. WINDER, S. J., HEMMINGS, L., MACIVER, S. K., BOLTON, S. J., TINSLEY, J. M., DAVIES, K. E., CRITCHLEY, D. R. & KENDRICK-JONES, J. (1995a) Utrophin actin binding domain: analysis of actin binding and cellular targeting. J. Cell Sci. 108, 63-71.
101. WINDER, S. J., GIBSON, T. J. & KENDRICK-JONES. J. (1995b) Dystrophin and utrophin: the missing links! FEBS Lett. 369, 27-33.
102. WINDER, S. J., GIBSON, T. J. & KENDRICK-JONES, J. (1996) Low probability of dystrophin and utrophin coiled coil regions forming dimers. Biochem. Soc. Trans. 24, 280S.
103. WINDER, S. J., KNIGHT, A. E. & KENDRICK-JONES, J. (1997) Protein Structure. In Dystrophin: Gene, Protein and Cell Biology (edited by LUCY, J. A. & BROWN, S. C.), pp. 27-55. Cambridge: Cambridge University Press.
104. WORTON, R. (1995) Muscular dystrophies: diseases of the dystrophin-glycoprotein complex. Science 270, 755-6.
105. YAMAMOTO, H., MIZUNO, Y., HAYASHI, K., NONAKA, I., YOSHIDA, M. & OZAWA, E. (1994) Expression of dystrophin-associated protein 35DAG (A4) and 50DAG (A2) is confined to striated muscles. J. Biochem. 115, 162-7.
106. YOSHIDA, M., SUZUKI, A., SHIMIZU, T. & OZAVVA, E. (1992) Proteinase sensitive sites on isolated rabbit dystrophin. J. Biochem. 112, 433-9.
107. YOSHIDA, M., SUZUKI, A., YAMAMOTO, H., NOGUCHI, S., MIZUNO, Y. & OZAWA, E. (1994) Dissociation of the complex of dystrophin and its associated proteins into several groups by n-octyl β-D-glucoside. Eur. J. Biochem. 222, 1055-61.
108. YOSHIDA, M., YAMAMOTO, H., NOGUCHI, S., MIZUNO, Y., HAGIWARA, Y. & OZAWA, E. (1995) Dystrophin-associated protein A0 is a homologue of the Torpedo 87K protein. FEBS Lett. 367, 311-4.