A-utrophin up-regulation in mdx skeletal muscle is independent of regeneration

Neuromuscular Disorders

Volumn 14, Issue 1, 2004, Pages 19-23

  Weir, Andrew P ^a   Morgan, Jennifer E ^a   Davies, Kay E ^a  

^a UNIVERSITY OF OXFORD   (United Kingdom)

Author keywords

Duchenne muscular dystrophy; Hypothesis; Utrophin

Indexed keywords

ISOPROTEIN; UNCLASSIFIED DRUG; UTROPHIN; UTROPHIN A; UTROPHIN B;

ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BINDING SITE; CONTROLLED STUDY; DUCHENNE MUSCULAR DYSTROPHY; GAMMA IRRADIATION; IMMUNOBLOTTING; IMMUNOHISTOCHEMISTRY; LEG MUSCLE; MOUSE; MUSCLE REGENERATION; NONHUMAN; PRIORITY JOURNAL; PROTEIN DEFICIENCY; PROTEIN EXPRESSION; SKELETAL MUSCLE; TRANSGENIC MOUSE; UPREGULATION;

EID: 0345118164     PISSN: 09608966     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.nmd.2003.09.004     Document Type: Article

References (37)

1. Love D.R., Hill D.F., Dickson G., et al. An autosomal transcript in skeletal muscle with homology to dystrophin. Nature. 339:1989;55-58.
2. Tinsley J.M., Blake D.J., Roche A., et al. Primary structure of dystrophin-related protein. Nature. 360:1992;591-593.
3. Matsumura K., Ervasti J.M., Ohlendieck K., Kahl S.D., Campbell K.P. Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle. Nature. 360:1992;588-591.
4. Winder S.J., Hemmings L., Maciver S.K., et al. Utrophin actin binding domain: analysis of actin binding and cellular targeting. J Cell Sci. 108:1995;63-71.
5. Roberts R.G., Bobrow M. Dystrophins in vertebrates and invertebrates. Hum Mol Genet. 7:1998;589-595.
6. Tinsley J.M., Davies K.E. Utrophin: a potential replacement for dystrophin? Neuromuscul Disord. 3:1993;537-539.
7. Kunkel L.M., Monaco A.P., Middlesworth W., Ochs H.D., Latt S.A. Specific cloning of DNA fragments absent from the DNA of a male patient with an X chromosome deletion. Proc Natl Acad Sci USA. 82:1985;4778-4782.
8. Monaco A.P., Neve R.L., Colletti-Feener C., et al. Isolation of candidate cDNAs for portions of the Duchenne muscular dystrophy gene. Nature. 323:1986;646-650.
9. Hoffman E.P., Brown R.H. Jr, Kunkel L.M. Dystrophin: the protein product of the Duchenne muscular dystrophy locus. Cell. 51:1987;919-928.
10. Tinsley J.M., Potter A.C., Phelps S.R., et al. Amelioration of the dystrophic phenotype of mdx mice using a truncated utrophin transgene [see comments]. Nature. 384:1996;349-353.
11. Tinsley J., Deconinck N., Fisher R., et al. Expression of full-length utrophin prevents muscular dystrophy in mdx mice. Nat Med. 4:1998;1441-1444.
12. Burton E.A., Tinsley J.M., Holzfeind P.J., Rodrigues N.R., Davies K.E. A second promoter provides an alternative target for therapeutic up-regulation of utrophin in Duchenne muscular dystrophy. Proc Natl Acad Sci USA. 96:1999;14025-14030.
13. Weir A.P., Burton E.A., Harrod G., Davies K.E. A- and B-utrophin have different expression patterns and are differentially up-regulated in mdx muscle. J Biol Chem. 277:2002;45285-45290.
14. 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. 180:1991;1179-1186.
15. Gramolini A.O., Karpati G., Jasmin B.J. Discordant expression of utrophin and its transcript in human and mouse skeletal muscles. J Neuropathol Exp Neurol. 58:1999;235-244.
16. Blake D.J., Weir A., Newey S.E., Davies K.E. Function and genetics of dystrophin and dystrophin-related proteins in muscle. Physiol Rev. 82:2002;291-329.
17. Infante J.P., Huszagh V.A. Mechanisms of resistance to pathogenesis in muscular dystrophies. Mol Cell Biochem. 195:1999;155-167.
18. Samarasinghe D.D. Some observations on the innervation of the striated muscle in the mouse oesophagus - an electron microscopy study. J Anat. 112:1972;173-184.
19. Sang Q., Young H.M. Development of nicotinic receptor clusters and innervation accompanying the change in muscle phenotype in the mouse esophagus. J Comp Neurol. 386:1997;119-136.
20. Boland B., Himpens B., Denef J.F., Gillis J.M. Site-dependent pathological differences in smooth muscles and skeletal muscles of the adult mdx mouse. Muscle Nerve. 18:1995;649-657.
21. Wakeford S., Watt D.J., Partridge T.A. X-irradiation improves mdx mouse muscle as a model of myofiber loss in DMD. Muscle Nerve. 14:1991;42-50.
22. Quinlan J.G., Cambier D., Lyden S., et al. Regeneration-blocked mdx muscle: in vivo model for testing treatments. Muscle Nerve. 20:1997;1016-1023.
23. Pagel C.N., Partridge T.A. Covert persistence of mdx mouse myopathy is revealed by acute and chronic effects of irradiation. J Neurol Sci. 164:1999;103-116.
24. Heslop L., Morgan J.E., Partridge T.A. Evidence for a myogenic stem cell that is exhausted in dystrophic muscle. J Cell Sci. 113:2000;2299-2308.
25. Weller B., Karpati G., Lehnert S., Carpenter S. Major alteration of the pathological phenotype in gamma irradiated mdx soleus muscles. J Neuropathol Exp Neurol. 50:1991;419-431.
26. Blake D.J., Hawkes R., Benson M.A., Beesley P.W. Different dystrophin-like complexes are expressed in neurons and glia. J Cell Biol. 147:1999;645-658.
27. Bulfield G., Siller W.G., Wight P.A., Moore K.J. X chromosome-linked muscular dystrophy (mdx) in the mouse. Proc Natl Acad Sci USA. 81:1984;1189-1192.
28. Quinlan J.G., Lyden S.P., Cambier D.M., et al. Radiation inhibition of mdx mouse muscle regeneration: dose and age factors. Muscle Nerve. 18:1995;201-206.
29. Loh N.Y., Newey S.E., Davies K.E., Blake D.J. Assembly of multiple dystrobrevin-containing complexes in the kidney. J Cell Sci. 113:2000;2715-2724.
30. Blake D.J., Nawrotzki R., Loh N.Y., Gorecki D.C., Davies K.E. Beta-dystrobrevin, a member of the dystrophin-related protein family. Proc Natl Acad Sci USA. 95:1998;241-246.
31. Patapoutian A., Wold B.J., Wagner R.A. Evidence for developmentally programmed transdifferentiation in mouse esophageal muscle. Science. 270:1995;1818-1821.
32. Stratton C.J., Bayguinov Y., Sanders K.M., Ward S.M. Ultrastructural analysis of the transdifferentiation of smooth muscle to skeletal muscle in the murine esophagus. Cell Tissue Res. 301:2000;283-298.
33. Zhao W., Dhoot G.K. Both smooth and skeletal muscle precursors are present in foetal mouse oesophagus and they follow different differentiation pathways. Dev Dyn. 218:2000;587-602.
34. Karpati G., Carpenter S., Morris G.E., et al. Localization and quantitation of the chromosome 6-encoded dystrophin-related protein in normal and pathological human muscle. J Neuropathol Exp Neurol. 52:1993;119-128.
35. Teijeira S., Teijeiro A., Fernandez R., Navarro C. Subsarcolemmal expression of utrophin in neuromuscular disorders: an immunohistochemical study of 80 cases. Acta Neuropathol (Berlin). 96:1998;481-486.
36. Lumeng C.N., Phelps S.F., Rafael J.A., et al. Characterization of dystrophin and utrophin diversity in the mouse. Hum Mol Genet. 8:1999;593-599.
37. Leibovitz S., Meshorer A., Fridman Y., et al. Exogenous Dp71 is a dominant negative competitor of dystrophin in skeletal muscle. Neuromuscul Disord. 12:2002;836-844.