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Items: 24


A gene-edited mouse model of Limb-Girdle muscular dystrophy 2C for testing exon skipping.

Demonbreun AR, Wyatt EJ, Fallon KS, Oosterbaan CC, Page P, Hadhazy M, Quattrocelli M, Barefield DY, McNally EM.

Dis Model Mech. 2019 Oct 3. pii: dmm.040832. doi: 10.1242/dmm.040832. [Epub ahead of print]


Deletion of Sulfonylurea Receptor 2 in the Adult Myocardium Enhances Cardiac Glucose Uptake and Is Cardioprotective.

Aubert G, Barefield DY, Demonbreun AR, Ramratnam M, Fallon KS, Warner JL, Rossi AE, Hadhazy M, Makielski JC, McNally EM.

JACC Basic Transl Sci. 2019 Apr 29;4(2):251-268. doi: 10.1016/j.jacbts.2018.11.012. eCollection 2019 Apr.


Dusp6 is a genetic modifier of growth through enhanced ERK activity.

Vo AH, Swaggart KA, Woo A, Gao QQ, Demonbreun AR, Fallon KS, Quattrocelli M, Hadhazy M, Page PGT, Chen Z, Eskin A, Squire K, Nelson SF, McNally EM.

Hum Mol Genet. 2019 Jan 15;28(2):279-289. doi: 10.1093/hmg/ddy349.


Genetic modifiers of muscular dystrophy act on sarcolemmal resealing and recovery from injury.

Quattrocelli M, Capote J, Ohiri JC, Warner JL, Vo AH, Earley JU, Hadhazy M, Demonbreun AR, Spencer MJ, McNally EM.

PLoS Genet. 2017 Oct 24;13(10):e1007070. doi: 10.1371/journal.pgen.1007070. eCollection 2017 Oct.


Experimental Modeling Supports a Role for MyBP-HL as a Novel Myofilament Component in Arrhythmia and Dilated Cardiomyopathy.

Barefield DY, Puckelwartz MJ, Kim EY, Wilsbacher LD, Vo AH, Waters EA, Earley JU, Hadhazy M, Dellefave-Castillo L, Pesce LL, McNally EM.

Circulation. 2017 Oct 17;136(16):1477-1491. doi: 10.1161/CIRCULATIONAHA.117.028585. Epub 2017 Aug 4.


Intermittent glucocorticoid steroid dosing enhances muscle repair without eliciting muscle atrophy.

Quattrocelli M, Barefield DY, Warner JL, Vo AH, Hadhazy M, Earley JU, Demonbreun AR, McNally EM.

J Clin Invest. 2017 Jun 1;127(6):2418-2432. doi: 10.1172/JCI91445. Epub 2017 May 8.


Overexpression of Latent TGFβ Binding Protein 4 in Muscle Ameliorates Muscular Dystrophy through Myostatin and TGFβ.

Lamar KM, Bogdanovich S, Gardner BB, Gao QQ, Miller T, Earley JU, Hadhazy M, Vo AH, Wren L, Molkentin JD, McNally EM.

PLoS Genet. 2016 May 5;12(5):e1006019. doi: 10.1371/journal.pgen.1006019. eCollection 2016 May.


Reengineering a transmembrane protein to treat muscular dystrophy using exon skipping.

Gao QQ, Wyatt E, Goldstein JA, LoPresti P, Castillo LM, Gazda A, Petrossian N, Earley JU, Hadhazy M, Barefield DY, Demonbreun AR, Bönnemann C, Wolf M, McNally EM.

J Clin Invest. 2015 Nov 2;125(11):4186-95. doi: 10.1172/JCI82768. Epub 2015 Oct 12.


Targeting latent TGFβ release in muscular dystrophy.

Ceco E, Bogdanovich S, Gardner B, Miller T, DeJesus A, Earley JU, Hadhazy M, Smith LR, Barton ER, Molkentin JD, McNally EM.

Sci Transl Med. 2014 Oct 22;6(259):259ra144. doi: 10.1126/scitranslmed.3010018.


Abcc9 is required for the transition to oxidative metabolism in the newborn heart.

Fahrenbach JP, Stoller D, Kim G, Aggarwal N, Yerokun B, Earley JU, Hadhazy M, Shi NQ, Makielski JC, McNally EM.

FASEB J. 2014 Jul;28(7):2804-15. doi: 10.1096/fj.13-244459. Epub 2014 Mar 19.


Dysferlin and myoferlin regulate transverse tubule formation and glycerol sensitivity.

Demonbreun AR, Rossi AE, Alvarez MG, Swanson KE, Deveaux HK, Earley JU, Hadhazy M, Vohra R, Walter GA, Pytel P, McNally EM.

Am J Pathol. 2014 Jan;184(1):248-59. doi: 10.1016/j.ajpath.2013.09.009. Epub 2013 Oct 29.


The superhealing MRL background improves muscular dystrophy.

Heydemann A, Swaggart KA, Kim GH, Holley-Cuthrell J, Hadhazy M, McNally EM.

Skelet Muscle. 2012 Dec 5;2(1):26. doi: 10.1186/2044-5040-2-26.


Cardiomyocyte sulfonylurea receptor 2-KATP channel mediates cardioprotection and ST segment elevation.

Stoller DA, Fahrenbach JP, Chalupsky K, Tan BH, Aggarwal N, Metcalfe J, Hadhazy M, Shi NQ, Makielski JC, McNally EM.

Am J Physiol Heart Circ Physiol. 2010 Oct;299(4):H1100-8. doi: 10.1152/ajpheart.00084.2010. Epub 2010 Jul 23.


Latent TGF-beta-binding protein 4 modifies muscular dystrophy in mice.

Heydemann A, Ceco E, Lim JE, Hadhazy M, Ryder P, Moran JL, Beier DR, Palmer AA, McNally EM.

J Clin Invest. 2009 Dec;119(12):3703-12. doi: 10.1172/JCI39845. Epub 2009 Nov 2. Erratum in: J Clin Invest. 2010 Feb;120(2):645.


Disruption of nesprin-1 produces an Emery Dreifuss muscular dystrophy-like phenotype in mice.

Puckelwartz MJ, Kessler E, Zhang Y, Hodzic D, Randles KN, Morris G, Earley JU, Hadhazy M, Holaska JM, Mewborn SK, Pytel P, McNally EM.

Hum Mol Genet. 2009 Feb 15;18(4):607-20. doi: 10.1093/hmg/ddn386. Epub 2008 Nov 13.


Nuclear sequestration of delta-sarcoglycan disrupts the nuclear localization of lamin A/C and emerin in cardiomyocytes.

Heydemann A, Demonbreun A, Hadhazy M, Earley JU, McNally EM.

Hum Mol Genet. 2007 Feb 15;16(4):355-63. Epub 2006 Dec 12.


Spontaneous coronary vasospasm in KATP mutant mice arises from a smooth muscle-extrinsic process.

Kakkar R, Ye B, Stoller DA, Smelley M, Shi NQ, Galles K, Hadhazy M, Makielski JC, McNally EM.

Circ Res. 2006 Mar 17;98(5):682-9. Epub 2006 Feb 2.


Normal myoblast fusion requires myoferlin.

Doherty KR, Cave A, Davis DB, Delmonte AJ, Posey A, Earley JU, Hadhazy M, McNally EM.

Development. 2005 Dec;132(24):5565-75. Epub 2005 Nov 9.


Genetic background influences muscular dystrophy.

Heydemann A, Huber JM, Demonbreun A, Hadhazy M, McNally EM.

Neuromuscul Disord. 2005 Oct;15(9-10):601-9.


Smooth muscle cell-extrinsic vascular spasm arises from cardiomyocyte degeneration in sarcoglycan-deficient cardiomyopathy.

Wheeler MT, Allikian MJ, Heydemann A, Hadhazy M, Zarnegar S, McNally EM.

J Clin Invest. 2004 Mar;113(5):668-75.


Cardiomyopathy is independent of skeletal muscle disease in muscular dystrophy.

Zhu X, Wheeler MT, Hadhazy M, Lam MY, McNally EM.

FASEB J. 2002 Jul;16(9):1096-8. Epub 2002 May 8.


Overexpression of gamma-sarcoglycan induces severe muscular dystrophy. Implications for the regulation of Sarcoglycan assembly.

Zhu X, Hadhazy M, Groh ME, Wheeler MT, Wollmann R, McNally EM.

J Biol Chem. 2001 Jun 15;276(24):21785-90. Epub 2001 Apr 3.


Differential requirement for individual sarcoglycans and dystrophin in the assembly and function of the dystrophin-glycoprotein complex.

Hack AA, Lam MY, Cordier L, Shoturma DI, Ly CT, Hadhazy MA, Hadhazy MR, Sweeney HL, McNally EM.

J Cell Sci. 2000 Jul;113 ( Pt 14):2535-44.


Dominant negative myostatin produces hypertrophy without hyperplasia in muscle.

Zhu X, Hadhazy M, Wehling M, Tidball JG, McNally EM.

FEBS Lett. 2000 May 26;474(1):71-5.

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