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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.


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.


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.


Alterations in Notch signalling in skeletal muscles from mdx and dko dystrophic mice and patients with Duchenne muscular dystrophy.

Church JE, Trieu J, Chee A, Naim T, Gehrig SM, Lamon S, Angelini C, Russell AP, Lynch GS.

Exp Physiol. 2014 Apr;99(4):675-87. doi: 10.1113/expphysiol.2013.077255. Epub 2014 Jan 17.


Skeletal Muscle Differentiation on a Chip Shows Human Donor Mesoangioblasts' Efficiency in Restoring Dystrophin in a Duchenne Muscular Dystrophy Model.

Serena E, Zatti S, Zoso A, Lo Verso F, Tedesco FS, Cossu G, Elvassore N.

Stem Cells Transl Med. 2016 Dec;5(12):1676-1683. Epub 2016 Aug 8.


Biochemical characterization of patients with in-frame or out-of-frame DMD deletions pertinent to exon 44 or 45 skipping.

Anthony K, Arechavala-Gomeza V, Ricotti V, Torelli S, Feng L, Janghra N, Tasca G, Guglieri M, Barresi R, Armaroli A, Ferlini A, Bushby K, Straub V, Ricci E, Sewry C, Morgan J, Muntoni F.

JAMA Neurol. 2014 Jan;71(1):32-40. doi: 10.1001/jamaneurol.2013.4908.


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.


Impaired regenerative capacity and lower revertant fibre expansion in dystrophin-deficient mdx muscles on DBA/2 background.

Rodrigues M, Echigoya Y, Maruyama R, Lim KR, Fukada SI, Yokota T.

Sci Rep. 2016 Dec 7;6:38371. doi: 10.1038/srep38371.


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.


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.


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.


Dystrophin quantification and clinical correlations in Becker muscular dystrophy: implications for clinical trials.

Anthony K, Cirak S, Torelli S, Tasca G, Feng L, Arechavala-Gomeza V, Armaroli A, Guglieri M, Straathof CS, Verschuuren JJ, Aartsma-Rus A, Helderman-van den Enden P, Bushby K, Straub V, Sewry C, Ferlini A, Ricci E, Morgan JE, Muntoni F.

Brain. 2011 Dec;134(Pt 12):3547-59. doi: 10.1093/brain/awr291. Epub 2011 Nov 18. Erratum in: Brain. 2016 Apr;139(Pt 4):e27.


Immunohistochemistry of sarcolemmal membrane-associated proteins in formalin-fixed and paraffin-embedded skeletal muscle tissue: a promising tool for the diagnostic evaluation of common muscular dystrophies.

Suriyonplengsaeng C, Dejthevaporn C, Khongkhatithum C, Sanpapant S, Tubthong N, Pinpradap K, Srinark N, Waisayarat J.

Diagn Pathol. 2017 Feb 20;12(1):19. doi: 10.1186/s13000-017-0610-y.

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