DOI: 10.1038/4033. Expression of full-length utrophin prevents muscular dystrophy in mdx mice

Expression of full-length utrophin prevents muscular dystrophy in mdx mice

10.1038/4033

Crossref journal-article

Springer Science and Business Media LLC

Nature Medicine (297)

Bibliography

Tinsley, J., Deconinck, N., Fisher, R., Kahn, D., Phelps, S., Gillis, J.-M., & Davies, K. (1998). Expression of full-length utrophin prevents muscular dystrophy in mdx mice. Nature Medicine, 4(12), 1441â1444.

Authors 7

Jonathon Tinsley (first)
Nicolas Deconinck (additional)
Rosie Fisher (additional)
David Kahn (additional)
Steve Phelps (additional)
Jean-Marie Gillis (additional)
Kay Davies (additional)

References 20 Referenced 500

Emery, A.E.H. in Duchenne Muscular Dystrophy (Oxford Medical Publications, Oxford, 1993). / Duchenne Muscular Dystrophy by AEH Emery (1993)
Tinsley, J.M. et al. Primary structure of dystrophin-related protein. Nature 360, 591–593 (1992). (10.1038/360591a0) / Nature by JM Tinsley (1992)
Love, D.R. et al. Tissue distribution of the dystrophin-related gene product and expression in the mdx and dy mouse. Proc. Natl. Acad. Sci. USA 88, 3243–3247 (1991). (10.1073/pnas.88.8.3243) / Proc. Natl. Acad. Sci. USA by DR Love (1991)
Khurana, T.S. et al. Immunolocalization and developmental expression of dystrophin related protein in skeletal muscle. Neuromuscul. Disord. 1, 185–194 (1991). (10.1016/0960-8966(91)90023-L) / Neuromuscul. Disord. by TS Khurana (1991)
Deconinck, N. et al. Expression of truncated utrophin leads to major functional improvements in dystrophin-deficient muscles of mice. Nature Med. 3, 1216–1221 (1997). (10.1038/nm1197-1216) / Nature Med. by N Deconinck (1997)
Tinsley, J.M. et al. Amelioration of the dystrophic phenotype of mdx mice using a truncated utrophin transgene. Nature 384, 349–353 (1996). (10.1038/384349a0) / Nature by JM Tinsley (1996)
Brennan, K.J. & Hardeman, E.C. Quantitative analysis of the human alpha-skeletal actin gene in transgenic mice. J. Biol. Chem. 268, 719–725 (1993). (10.1016/S0021-9258(18)54211-3) / J. Biol. Chem. by KJ Brennan (1993)
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, 1189–1192 (1984). (10.1073/pnas.81.4.1189) / Proc. Natl. Acad. Sci. USA by G Bulfield (1984)
Blake, D.J. et al. G-utrophin, the autosomal homologue of dystrophin Dp116, is expressed in sensory ganglia and brain. Proc. Natl. Acad. Sc.i USA 92, 3697–3701 (1995). (10.1073/pnas.92.9.3697) / Proc. Natl. Acad. Sc.i USA by DJ Blake (1995)
Straub, V. & Campbell, K.P. Muscular dystrophies and the dystrophin-glycoprotein complex. Curr. Opin. Neurol. 10, 168–175 (1997). (10.1097/00019052-199704000-00016) / Curr. Opin. Neurol. by V Straub (1997)
Sadoulet, P.H., Rajala, M. & Kunkel, L.M. Dystrobrevin and dystrophin: an interaction through coiled-coil motifs. Proc. Natl. Acad. Sci. USA 94, 12413–12418 (1997). (10.1073/pnas.94.23.12413) / Proc. Natl. Acad. Sci. USA by PH Sadoulet (1997)
Suzuki, A., Yoshida, M. & Ozawa, E. Mammalian alpha 1- and beta 1-syntrophin bind to the alternative splice-prone region of the dystrophin COOH terminus. J. Cell. Biol. 128, 373–381 (1995). (10.1083/jcb.128.3.373) / J. Cell. Biol. by A Suzuki (1995)
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, 241–246 (1998). (10.1073/pnas.95.1.241) / Proc. Natl. Acad. Sci. USA by DJ Blake (1998)
Moens, P., Baatsen, P.H. & Marechal, G. Increased susceptibility of EDL muscles from mdx mice to damage induced by contractions with stretch. J. Muscle Res. Cell. Motil. 14, 446–451 (1993). (10.1007/BF00121296) / J. Muscle Res. Cell. Motil. by P Moens (1993)
Petrof, B.J., Shrager, J.B., Stedman, H.H., Kelly, A.M. & Sweeney, H.L. Dystrophin protects the sarcolemma from stresses developed during muscle contraction. Proc. Natl. Acad. Sci. USA 90, 3710–3714 (1993). (10.1073/pnas.90.8.3710) / Proc. Natl. Acad. Sci. USA by BJ Petrof (1993)
Weller, B., Karpati, G. & Carpenter, S. Dystrophin-deficient mdx muscle fibers are preferentially vulnerable to necrosis induced by experimental lengthening contractions. J. Neurol. Sci. 100, 9–13 (1990). (10.1016/0022-510X(90)90005-8) / J. Neurol. Sci. by B Weller (1990)
Stedman, H.H. et al. The mdx mouse diaphragm reproduces the degenerative changes of Duchenne muscular dystrophy. Nature 352, 536–539 (1991). (10.1038/352536a0) / Nature by HH Stedman (1991)
Nguyen, T.M. et al. 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. J. Cell. Biol. 115, 1695–1700 (1991). (10.1083/jcb.115.6.1695) / J. Cell. Biol. by TM Nguyen (1991)
Carlson, C.G. & Makiejus, R.V. A non-invassive procedure to detect muscle weakness in the mdx mouse. Muscle Nerve 13, 480–484 (1990). (10.1002/mus.880130603) / Muscle Nerve by CG Carlson (1990)
Deconinck, A.E. et al. Postsynaptic abnormalities at the neuromuscular junctions of utrophin-deficient mice. J. Cell. Biol. 136, 88–894 (1997). (10.1083/jcb.136.4.883) / J. Cell. Biol. by AE Deconinck (1997)

Dates

Type	When
Created	23 years, 1 month ago (July 26, 2002, 4:43 a.m.)
Deposited	2 years, 3 months ago (May 18, 2023, 6:24 p.m.)
Indexed	1 week, 2 days ago (Aug. 21, 2025, 1:21 p.m.)
Issued	26 years, 8 months ago (Dec. 1, 1998)
Published	26 years, 8 months ago (Dec. 1, 1998)
Published Print	26 years, 8 months ago (Dec. 1, 1998)

Funders 0

None

BibTeX

@article{Tinsley_1998, title={Expression of full-length utrophin prevents muscular dystrophy in mdx mice}, volume={4}, ISSN={1546-170X}, url={http://dx.doi.org/10.1038/4033}, DOI={10.1038/4033}, number={12}, journal={Nature Medicine}, publisher={Springer Science and Business Media LLC}, author={Tinsley, Jonathon and Deconinck, Nicolas and Fisher, Rosie and Kahn, David and Phelps, Steve and Gillis, Jean-Marie and Davies, Kay}, year={1998}, month=dec, pages={1441–1444} }

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