Dystrophin, utrophin and β-dystroglycan expression in skeletal muscle from patients with Becker muscular dystrophy

Journal of Neuropathology and Experimental Neurology

Volumn 55, Issue 8, 1996, Pages 896-903

  Kawajiri, Masakazu ^a   Mitsui, Takao ^a   Kawai, Hisaomi ^a   Kobunai, Takashi ^b   Tsuchihashi, Takayuki ^a   Saito, Shiro ^a  

^a UNIVERSITY OF TOKUSHIMA   (Japan)

^b TAIHO PHARMACEUTICAL CO LTD   (Japan)

Author keywords

Becker muscular dystrophy; Confocal laser microscopy; Dystrophin; Utrophin; dystroglycan

Indexed keywords

BETA DYSTROGLYCAN; DYSTROPHIN; MEMBRANE PROTEIN; MONOCLONAL ANTIBODY; UNCLASSIFIED DRUG; UTROPHIN;

ADOLESCENT; ADULT; BECKER MUSCULAR DYSTROPHY; CHILD; CLINICAL ARTICLE; CONFOCAL LASER MICROSCOPY; CONTROLLED STUDY; FLUORESCENCE MICROSCOPY; HUMAN; HUMAN TISSUE; IMMUNOFLUORESCENCE TEST; IMMUNOHISTOCHEMISTRY; MUSCLE BIOPSY; PRIORITY JOURNAL; PROTEIN BINDING; PROTEIN EXPRESSION; REVIEW; SARCOLEMMA; SKELETAL MUSCLE;

EID: 0029818883     PISSN: 00223069     EISSN: None     Source Type: Journal    
DOI: 10.1097/00005072-199608000-00005     Document Type: Review

References (35)

1. Hoffman EP, Brown RH Jr., Kunkel LM. Dystrophin: The protein product of the Duchenne muscular dystrophy locus. Cell 1987;51:919-28
2. Koenig M, Monaco AP, Kunkel LM. The complete sequence of dystrophin predicts a rod-shaped cytoskeletal protein. Cell 1988;53:219-28
3. Ervasti JM, Campbell KP. Membrane organization of the dystrophin-glycoprotein complex. Cell 1991;66:1121-31
4. Ibraghimov-Beskrovnaya O, Ervasti JM, Leveille CJ, Slaughter CA, Sernett SW, Campbell KP. Primary structure of dystrophinassociated glycoproteins linking dystrophin to the extracellular matrix. Nature 1992;355:696-702
5. Campbell KP. Three muscular dystrophies: Loss of cytoskeleton extracellular matrix linkage. Cell 1995;80:675-79
6. Ervasti JM, Campbell KP. A role for the dystrophin-glycoprotein complex as a transmembrane linker between laminin and actin. J Cell Biol 1993;122:809-23
7. Suzuki A, Yoshida M, Yamamoto H, Ozawa E. Glycoprotein-binding site of dystrophin is confined to the cystein-rich domain and the first half of the carboxy-terminal domain. FEBS Lett 1992;308:154-60
8. Tanaka H, Ishiguro T, Eguchi C, Saito K, Ozawa E. Expression of a dystrophin-related protein aasociated with the skeletal muscle cell membrane. Histochemistry 1991;96:1-5
9. Nguyen TM, Ellis JM, Love DR, Davies KE, Gatter KC, Dickson G, Morris GE. Localization of the DMDL-gene-encoded dystrophin-related protein using a panel of nineteen monoclonal antibodies. J Cell Biol 1991;115:1695-1700
10. Ohlendieck K, Ervasti JM, Matsumura K, Kahl SD, Leveille CJ, Campbell KP. Dystrophin-related protein is localized to neuromuscular junctions of adult skeletal muscle. Neuron 1991;7:499-508
11. Takemitsu M, Ishiura S, Koga R, et al. Dystrophin-related protein in the fetal and denervated skeletal muscles of normal and mdx mice. Biochem Biophys Res Commun 1991;180:1179-86
12. Takemitsu M, Nonaka I, Sugita H. Dystrophin-related protein in skeletal muscles in neuromuscular disorders: Immunohistochemical study. Acta Neuropath 1993;85:1179-86
13. Campanelli JT, Roberds SL, Campbell KP, Scheller RH. A role for dystrophin-associated glycoproteins and utrophin in agrin-induced AChR clustering. Cell 1994;77:663-74
14. Gee SH, Montanaro F, Lindenbaum MH, Carbonetto S. Dystroglycan-α, a dystrophin-associated glycoprotein, is a functional agrin receptor. Cell 1994;77:675-86
15. Engel AG, Yamamoto M, Fischbeck KH. Dystrophinopathies. In: Engel AG, Franzini-Armstrong C, eds. Myology, second edition. New York: McGraw-Hill, 1994:1133-87
16. Matsumura K, Nonaka I, Tome FMS, et al. Mild deficiency of dystrophin-associated proteins having in-frame deletions in the rod domain of dystrophin. Am J Hum Genet 1993;53:709-16
17. Mizuno Y, Yoshida M, Nonaka I, Hirai S, Ozawa E. Expression of utrophin (dystrophin-related protein) and dystrophin-associated glycoproteins in muscles from patients with Duchenne muscular dystrophy. Muscle Nerve 1994;17:206-16
18. Mizuno Y, Nonaka I, Hirai S, Ozawa E. Reciprocal expression of dystrophin and utrophin in muscles of Duchenne muscular dystrophy patients, female DMD-carriers and control subjects. J Neurol Sci 1993;119:43-52
19. Beggs AH, Kunkel LM. Improved diagnosis of Duchenne/Becker muscular dystrophy. J Clin Invest 1990;85:613-19
20. Chamberlain JS, Farwell NJ, Chamberlain JR, Cox GA, Caskey CT. PCR analyses of dystrophin gene mutation and expression. J Cell Biochem 1991;46:255-59
21. Tanaka H, Ozawa E. Expression of dystrophin mRNA and the protein in the developing rat heart. Biochem Biophys Res Commun 1990;172:824-29
22. Lidov H, Byer TJ, Watkins SC, Kunkel LM. Localization of dystrophin to postsynaptic regions of central nervous system cortical neurons. Nature 1990;348:725-28
23. Hagiwara Y, Yoshida M, Nonaka I, Ozawa E. Developmental expression of dystrophin on the plasma membrane of rat muscle cells. Protoplasma 1989;151:11-18
24. Doucet JP, Trifaro JM. A discontinuous and highly porous sodium dodecyl sulfate-polyacrylamide slab gel system of high resolution. Anal Biochem 1988;168:265-71
25. Kyshe-Anderson J. Electroblotting of multiple gels: A simple apparatus without buffer tank for rapid transfer of proteins from polyacrylamide to nitrocellulose. J Biochem Biophys Methods 1984;10:203-9
26. Bolton S. Pharmaceutical statistics. New York: Marcel Deckker Inc. 1984
27. White JC, Amos WB, Fordham M. An evaluation of confocal versus conventional imaging of biological structures by fluorescence light microscopy. J Cell Biol 1987;105:41-48
28. Masuda T, Fujimaki N, Ozawa E, Ishikawa H. Confocal laser microscopy of dystrophin localization in guinea pig skeletal muscle fibers. J Cell Biol 1992;119:543-48
29. Pons F, Nicholson LVB, Robert A, Voit T, Leger JJ. Dystrophin and dystrophin-related protein (utrophin) distribution in normal and dystrophin-deficient skeletal muscles. Neuromuscul Disord 1993;3:507-14
30. Lakkakorpi JT, Rajaniemi HJ. Application of the immunofluorescence technique and confocal laser scanning microscopy for studying the distribution of the luteinizing hormone/chorionic gonadotropin (LH/CG) receptor of rat luteal cells. J Histochem Cytochem 1991;39:397-400
31. Porter GA, Dmytrenko GM, Winkelmann JC, Bloch RJ. Dystrophin colocalizes with β-spectrin in distinct subsarcolemmal domains in mammalian skeletal muscle. J Cell Biol 1992;117:997-1005
32. Apel ED, Roberds SL, Campbell KP, Merlie JP. Rapsyn may function as a link between the acetylcholine receptor and the agrinbinding dystrophin-associated glycoprotein complex. Neuron 1995;15:115-26
33. Tinsley JM, Blake DJ, Roche A, et al. Primary structure of dystrophinrelated protein. Nature 1992;360:591-93
34. Matsumura K, Ervasti JM, Ohlendieck K, Kahl SD, Campbell KP. Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle. Nature 1992;360:588-91
35. Mitsui T, Tsuchihashi T, Ueta Y, Kawai H, Saito S. Expression of genes for dystrophin, dystrophin-associated glycoprotein and dystrophin-related protein in skeletal muscle of normal subjects and patients with Duchenne/Becker muscular dystrophy. Muscle Nerve 1994; Supplement 1:S99