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Revertant fibres and dystrophin traces in Duchenne muscular dystrophy: implication for clinical trials.

Arechavala-Gomeza V, Kinali M, Feng L, Guglieri M, Edge G, Main M, Hunt D, Lehovsky J, Straub V, Bushby K, Sewry CA, Morgan JE, Muntoni F.

Neuromuscul Disord. 2010 May;20(5):295-301. doi: 10.1016/j.nmd.2010.03.007. Epub 2010 Apr 14.


Correlation of Utrophin Levels with the Dystrophin Protein Complex and Muscle Fibre Regeneration in Duchenne and Becker Muscular Dystrophy Muscle Biopsies.

Janghra N, Morgan JE, Sewry CA, Wilson FX, Davies KE, Muntoni F, Tinsley J.

PLoS One. 2016 Mar 14;11(3):e0150818. doi: 10.1371/journal.pone.0150818. eCollection 2016.


Revertant fibers in the mdx murine model of Duchenne muscular dystrophy: an age- and muscle-related reappraisal.

Pigozzo SR, Da Re L, Romualdi C, Mazzara PG, Galletta E, Fletcher S, Wilton SD, Vitiello L.

PLoS One. 2013 Aug 28;8(8):e72147. doi: 10.1371/journal.pone.0072147. eCollection 2013.


A sensitive, reproducible and objective immunofluorescence analysis method of dystrophin in individual fibers in samples from patients with duchenne muscular dystrophy.

Beekman C, Sipkens JA, Testerink J, Giannakopoulos S, Kreuger D, van Deutekom JC, Campion GV, de Kimpe SJ, Lourbakos A.

PLoS One. 2014 Sep 22;9(9):e107494. doi: 10.1371/journal.pone.0107494. eCollection 2014.


Transgenic overexpression of caveolin-3 in skeletal muscle fibers induces a Duchenne-like muscular dystrophy phenotype.

Galbiati F, Volonte D, Chu JB, Li M, Fine SW, Fu M, Bermudez J, Pedemonte M, Weidenheim KM, Pestell RG, Minetti C, Lisanti MP.

Proc Natl Acad Sci U S A. 2000 Aug 15;97(17):9689-94.


L-type Ca2+ channel function is linked to dystrophin expression in mammalian muscle.

Friedrich O, von Wegner F, Chamberlain JS, Fink RH, Rohrbach P.

PLoS One. 2008 Mar 12;3(3):e1762. doi: 10.1371/journal.pone.0001762.


Proteasome inhibitor (MG-132) treatment of mdx mice rescues the expression and membrane localization of dystrophin and dystrophin-associated proteins.

Bonuccelli G, Sotgia F, Schubert W, Park DS, Frank PG, Woodman SE, Insabato L, Cammer M, Minetti C, Lisanti MP.

Am J Pathol. 2003 Oct;163(4):1663-75.


Utility of dystrophin and utrophin staining in childhood muscular dystrophy.

Sundaram C, Vydehi B, Meena K, Murthy J.

Indian J Pathol Microbiol. 2004 Jul;47(3):367-9.


Pharmacological rescue of the dystrophin-glycoprotein complex in Duchenne and Becker skeletal muscle explants by proteasome inhibitor treatment.

Assereto S, Stringara S, Sotgia F, Bonuccelli G, Broccolini A, Pedemonte M, Traverso M, Biancheri R, Zara F, Bruno C, Lisanti MP, Minetti C.

Am J Physiol Cell Physiol. 2006 Feb;290(2):C577-82. Epub 2005 Sep 28.


Suppression of revertant fibers in mdx mice by expression of a functional dystrophin.

Crawford GE, Lu QL, Partridge TA, Chamberlain JS.

Hum Mol Genet. 2001 Nov 15;10(24):2745-50.


The role of branched fibres in the pathogenesis of Duchenne muscular dystrophy.

Chan S, Head SI.

Exp Physiol. 2011 Jun;96(6):564-71. doi: 10.1113/expphysiol.2010.056713. Epub 2011 Mar 18. Review.


Brain function in Duchenne muscular dystrophy.

Anderson JL, Head SI, Rae C, Morley JW.

Brain. 2002 Jan;125(Pt 1):4-13. Review.


Enhanced expression of the alpha 7 beta 1 integrin reduces muscular dystrophy and restores viability in dystrophic mice.

Burkin DJ, Wallace GQ, Nicol KJ, Kaufman DJ, Kaufman SJ.

J Cell Biol. 2001 Mar 19;152(6):1207-18.


Long-term persistence of donor nuclei in a Duchenne muscular dystrophy patient receiving bone marrow transplantation.

Gussoni E, Bennett RR, Muskiewicz KR, Meyerrose T, Nolta JA, Gilgoff I, Stein J, Chan YM, Lidov HG, Bönnemann CG, Von Moers A, Morris GE, Den Dunnen JT, Chamberlain JS, Kunkel LM, Weinberg K.

J Clin Invest. 2002 Sep;110(6):807-14.


Temporal gene expression profiling of dystrophin-deficient (mdx) mouse diaphragm identifies conserved and muscle group-specific mechanisms in the pathogenesis of muscular dystrophy.

Porter JD, Merriam AP, Leahy P, Gong B, Feuerman J, Cheng G, Khanna S.

Hum Mol Genet. 2004 Feb 1;13(3):257-69. Epub 2003 Dec 17.


Dystrophin analysis in carriers of Duchenne and Becker muscular dystrophy.

Hoogerwaard EM, Ginjaar IB, Bakker E, de Visser M.

Neurology. 2005 Dec 27;65(12):1984-6.


Physiological characterization of muscle strength with variable levels of dystrophin restoration in mdx mice following local antisense therapy.

Sharp PS, Bye-a-Jee H, Wells DJ.

Mol Ther. 2011 Jan;19(1):165-71. doi: 10.1038/mt.2010.213. Epub 2010 Oct 5.


Aciculin and its relation to dystrophin: immunocytochemical studies in human normal and Duchenne dystrophy quadriceps muscles.

Wakayama Y, Inoue M, Kojima H, Murahashi M, Shibuya S, Yamashita S, Oniki H.

Acta Neuropathol. 2000 Jun;99(6):654-62.

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